Journal articles: 'Mitochondrial medicine'

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Weissig, Volkmar, and Marvin Edeas. "Recent developments in mitochondrial medicine (Part 1)." 4open 4 (2021): 2. http://dx.doi.org/10.1051/fopen/2021002.

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Research into elucidating structure and function of mitochondria has been quite steady between the time of discovery during the end of the 19th century until towards the late 1980’s. During the 1990s there was talk about a “comeback” of this organelle reflecting a widely revitalized interest into mitochondrial research which was based on two major discoveries made during that time. The first was the etiological association between human diseases and mitochondrial DNA mutations, while the second revealed the crucial function of mitochondria during apoptosis. The March 5th, 1999 issue of Science even featured a textbook image of a mitochondrion on its front cover and was entirely dedicated to this organelle. Whilst the term “comeback” might have been appropriate to describe the general excitement surrounding the new mitochondrial discoveries made during the 1990s, a term for describing the progress made in mitochondrial research during the last two decades is difficult to find. Between 2000 and 2020 the number of publications on mitochondria has skyrocketed. It is now widely accepted that there hardly exists any human disease for which either the etiology or pathogenesis does not seem to be associated with mitochondrial malfunction. In this review we will discuss and follow several lines of mitochondrial research from their early beginnings up to the present. We hope to be able to convince the reader of what we expressed about a decade ago, that the future of medicine will come through mitochondria.

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Weissig, Volkmar, and Marvin Edeas. "Recent developments in mitochondrial medicine (part 2)." 4open 5 (2022): 5. http://dx.doi.org/10.1051/fopen/2022002.

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Called “bioblasts” in 1890, named “mitochondria” in 1898, baptized in 1957 as the “powerhouse of the cell” and christened in 1999 as the “motor of cell death”, mitochondria have been anointed in 2017 as “powerhouses of immunity”. In 1962, for the first time a causal link between mitochondria and human diseases was described, the genetic basis for which was revealed in 1988. The term “mitochondrial medicine” was coined in 1994. Research into mitochondria has been conducted ever since light microscopic studies during the end of the 19th century revealed their existence. To this day, new discoveries around this organelle and above all new insights into their fundamental role for human health and disease continue to surprise. Nowadays hardly any disease is known for which either the etiology or pathogenesis is not associated with malfunctioning mitochondria. In this second part of our review about recent developments in mitochondrial medicine we continue tracking and highlighting selected lines of mitochondrial research from their beginnings up to the present time. Mainly written for readers not familiar with this cell organelle, we hope both parts of our review will substantiate what we articulated over a decade ago, namely that the future of medicine will come through better understanding of the mitochondrion.

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D’Amato, Marco, Francesca Morra, Ivano Di Di Meo, and Valeria Tiranti. "Mitochondrial Transplantation in Mitochondrial Medicine: Current Challenges and Future Perspectives." International Journal of Molecular Sciences 24, no. 3 (January 19, 2023): 1969. http://dx.doi.org/10.3390/ijms24031969.

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Mitochondrial diseases (MDs) are inherited genetic conditions characterized by pathogenic mutations in nuclear DNA (nDNA) or mitochondrial DNA (mtDNA). Current therapies are still far from being fully effective and from covering the broad spectrum of mutations in mtDNA. For example, unlike heteroplasmic conditions, MDs caused by homoplasmic mtDNA mutations do not yet benefit from advances in molecular approaches. An attractive method of providing dysfunctional cells and/or tissues with healthy mitochondria is mitochondrial transplantation. In this review, we discuss what is known about intercellular transfer of mitochondria and the methods used to transfer mitochondria both in vitro and in vivo, and we provide an outlook on future therapeutic applications. Overall, the transfer of healthy mitochondria containing wild-type mtDNA copies could induce a heteroplasmic shift even when homoplasmic mtDNA variants are present, with the aim of attenuating or preventing the progression of pathological clinical phenotypes. In summary, mitochondrial transplantation is a challenging but potentially ground-breaking option for the treatment of various mitochondrial pathologies, although several questions remain to be addressed before its application in mitochondrial medicine.

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Wang, Jie, Fei Lin, Li-li Guo, Xing-jiang Xiong, and Xun Fan. "Cardiovascular Disease, Mitochondria, and Traditional Chinese Medicine." Evidence-Based Complementary and Alternative Medicine 2015 (2015): 1–7. http://dx.doi.org/10.1155/2015/143145.

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Recent studies demonstrated that mitochondria play an important role in the cardiovascular system and mutations of mitochondrial DNA affect coronary artery disease, resulting in hypertension, atherosclerosis, and cardiomyopathy. Traditional Chinese medicine (TCM) has been used for thousands of years to treat cardiovascular disease, but it is not yet clear how TCM affects mitochondrial function. By reviewing the interactions between the cardiovascular system, mitochondrial DNA, and TCM, we show that cardiovascular disease is negatively affected by mutations in mitochondrial DNA and that TCM can be used to treat cardiovascular disease by regulating the structure and function of mitochondria via increases in mitochondrial electron transport and oxidative phosphorylation, modulation of mitochondrial-mediated apoptosis, and decreases in mitochondrial ROS. However further research is still required to identify the mechanism by which TCM affects CVD and modifies mitochondrial DNA.

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Kiseljaković, Emina, Radivoj Jadrić, Sabaheta Hasić, Lorenka Ljuboja, Jovo Radovanović, Husein Kulenović, and Mira Winterhalter-Jadrić. "Mitochondrial medicine - a key to solve pathophysiology of 21 century diseases." Bosnian Journal of Basic Medical Sciences 2, no. 1-2 (February 20, 2008): 46–48. http://dx.doi.org/10.17305/bjbms.2002.3580.

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Over the past 13 years mitochondrial defects have been involved in wide variety of degenerative diseases - Parkinson disease, Alzheimer dementia, arteriosclerosis, ageing and cancer. Mitochondria are believed to control apoptosis or programmed cell death. Disturbance in mitochondrial metabolism has also been implicated in many common diseases such as congestive hart failure, diabetes and migraine. Scientific investigations have showed complexities in mitochondrial genetics, but at the same time, pathophysiology of mitochondrial diseases is still enigma. Mitochondria and their DNAs are opening the era of "mitochondrial medicine". What we today call "a mitochondrial medicine" is only a part of the whole panorama of diseases based on disordered mitochondrial function.

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Qin, Lingyu, and Shuhua Xi. "The role of Mitochondrial Fission Proteins in Mitochondrial Dynamics in Kidney Disease." International Journal of Molecular Sciences 23, no. 23 (November 25, 2022): 14725. http://dx.doi.org/10.3390/ijms232314725.

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Mitochondria have many forms and can change their shape through fusion and fission of the outer and inner membranes, called “mitochondrial dynamics”. Mitochondrial outer membrane proteins, such as mitochondrial fission protein 1 (FIS1), mitochondrial fission factor (MFF), mitochondrial 98 dynamics proteins of 49 kDa (MiD49), and mitochondrial dynamics proteins of 51 kDa (MiD51), can aggregate at the outer mitochondrial membrane and thus attract Dynamin-related protein 1 (DRP1) from the cytoplasm to the outer mitochondrial membrane, where DRP1 can perform a scissor-like function to cut a complete mitochondrion into two separate mitochondria. Other organelles can promote mitochondrial fission alongside mitochondria. FIS1 plays an important role in mitochondrial–lysosomal contacts, differentiating itself from other mitochondrial-fission-associated proteins. The contact between the two can also induce asymmetric mitochondrial fission. The kidney is a mitochondria-rich organ, requiring large amounts of mitochondria to produce energy for blood circulation and waste elimination. Pathological increases in mitochondrial fission can lead to kidney damage that can be ameliorated by suppressing their excessive fission. This article reviews the current knowledge on the key role of mitochondrial-fission-associated proteins in the pathogenesis of kidney injury and the role of their various post-translational modifications in activation or degradation of fission-associated proteins and targeted drug therapy.

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Michelakis, Evangelos D. "Mitochondrial Medicine." Circulation 117, no. 19 (May 13, 2008): 2431–34. http://dx.doi.org/10.1161/circulationaha.108.775163.

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Chinnery, P. F., and D. M. Turnbull. "Mitochondrial medicine." QJM 90, no. 11 (November 1, 1997): 657–67. http://dx.doi.org/10.1093/qjmed/90.11.657.

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Dorey, Emma. "Mitochondrial medicine." Nature Biotechnology 32, no. 4 (April 2014): 300. http://dx.doi.org/10.1038/nbt0414-300a.

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Luft, Rolf, and B. R. LANDAU. "Mitochondrial medicine." Journal of Internal Medicine 238, no. 5 (November 1995): 405–21. http://dx.doi.org/10.1111/j.1365-2796.1995.tb01218.x.

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DiMauro, Salvatore. "Mitochondrial medicine." Biochimica et Biophysica Acta (BBA) - Bioenergetics 1659, no. 2-3 (December 2004): 107–14. http://dx.doi.org/10.1016/j.bbabio.2004.08.003.

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Koene, Saskia, and Jan Smeitink. "Mitochondrial medicine." Journal of Inherited Metabolic Disease 34, no. 2 (March 2, 2011): 247–48. http://dx.doi.org/10.1007/s10545-011-9292-x.

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Rosenberg, Roger N. "Mitochondrial Medicine." Archives of Neurology 63, no. 10 (October 1, 2006): 1505. http://dx.doi.org/10.1001/archneur.63.10.1505.

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Yamada, Yuma, and Hideyoshi Harashima. "Targeting Mitochondria: Innovation from Mitochondrial Drug Delivery System (DDS) to Mitochondrial Medicine." YAKUGAKU ZASSHI 132, no. 10 (October 1, 2012): 1111–18. http://dx.doi.org/10.1248/yakushi.12-00220-4.

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Lin, Tsu-Kung, Shang-Der Chen, Yao-Chung Chuang, Min-Yu Lan, Jiin-Haur Chuang, Pei-Wen Wang, Te-Yao Hsu, et al. "Mitochondrial Transfer of Wharton’s Jelly Mesenchymal Stem Cells Eliminates Mutation Burden and Rescues Mitochondrial Bioenergetics in Rotenone-Stressed MELAS Fibroblasts." Oxidative Medicine and Cellular Longevity 2019 (May 22, 2019): 1–17. http://dx.doi.org/10.1155/2019/9537504.

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Wharton’s jelly mesenchymal stem cells (WJMSCs) transfer healthy mitochondria to cells harboring a mitochondrial DNA (mtDNA) defect. Mitochondrial myopathy, encephalomyopathy, lactic acidosis, and stroke-like episodes (MELAS) is one of the major subgroups of mitochondrial diseases, caused by the mt.3243A>G point mutation in the mitochondrial tRNALeu(UUR) gene. The specific aim of the study is to investigate whether WJMSCs exert therapeutic effect for mitochondrial dysfunction in cells of MELAS patient through donating healthy mitochondria. We herein demonstrate that WJMSCs transfer healthy mitochondria into rotenone-stressed fibroblasts of a MELAS patient, thereby eliminating mutation burden and rescuing mitochondrial functions. In the coculture system in vitro study, WJMSCs transferred healthy mitochondria to rotenone-stressed MELAS fibroblasts. By inhibiting actin polymerization to block tunneling nanotubes (TNTs), the WJMSC-conducted mitochondrial transfer was abrogated. After mitochondrial transfer, the mt.3243A>G mutation burden of MELAS fibroblasts was reduced to an undetectable level, with long-term retention. Sequencing results confirmed that the transferred mitochondria were donated from WJMSCs. Furthermore, mitochondrial transfer of WJMSCs to MELAS fibroblasts improves mitochondrial functions and cellular performance, including protein translation of respiratory complexes, ROS overexpression, mitochondrial membrane potential, mitochondrial morphology and bioenergetics, cell proliferation, mitochondrion-dependent viability, and apoptotic resistance. This study demonstrates that WJMSCs exert bioenergetic therapeutic effects through mitochondrial transfer. This finding paves the way for the development of innovative treatments for MELAS and other mitochondrial diseases.

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Murata, Daisuke, Kenta Arai, Miho Iijima, and Hiromi Sesaki. "Mitochondrial division, fusion and degradation." Journal of Biochemistry 167, no. 3 (December 4, 2019): 233–41. http://dx.doi.org/10.1093/jb/mvz106.

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Abstract The mitochondrion is an essential organelle for a wide range of cellular processes, including energy production, metabolism, signal transduction and cell death. To execute these functions, mitochondria regulate their size, number, morphology and distribution in cells via mitochondrial division and fusion. In addition, mitochondrial division and fusion control the autophagic degradation of dysfunctional mitochondria to maintain a healthy population. Defects in these dynamic membrane processes are linked to many human diseases that include metabolic syndrome, myopathy and neurodegenerative disorders. In the last several years, our fundamental understanding of mitochondrial fusion, division and degradation has been significantly advanced by high resolution structural analyses, protein-lipid biochemistry, super resolution microscopy and in vivo analyses using animal models. Here, we summarize and discuss this exciting recent progress in the mechanism and function of mitochondrial division and fusion.

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Wang, Zhe, Hai Bo, Yu Song, Can Li, and Yong Zhang. "Mitochondrial ROS Produced by Skeletal Muscle Mitochondria Promote the Decisive Signal for UPRmt Activation." BioMed Research International 2022 (February 21, 2022): 1–8. http://dx.doi.org/10.1155/2022/7436577.

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The mitochondrial unfolded protein response (UPRmt) can repair and remove misfolded or unfolded proteins in mitochondria and enhance mitochondrial protein homeostasis. Reactive oxygen species (ROS) produced by regular exercise is a crucial signal for promoting health, and skeletal muscle mitochondria are the primary source of ROS during exercise. To verify whether UPRmt is related to ROS produced by mitochondria in skeletal muscle during regular exercise, we adapted MitoTEMPO, mitochondrially targeted antioxidants, and ROS production by mitochondria. Our results showed that mitochondrial ROS is the key factor for activating UPRmt in different pathways.

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Zorov, Dmitry B., Magdalena Juhaszova, and Steven J. Sollott. "Mitochondrial Reactive Oxygen Species (ROS) and ROS-Induced ROS Release." Physiological Reviews 94, no. 3 (July 2014): 909–50. http://dx.doi.org/10.1152/physrev.00026.2013.

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Byproducts of normal mitochondrial metabolism and homeostasis include the buildup of potentially damaging levels of reactive oxygen species (ROS), Ca2+, etc., which must be normalized. Evidence suggests that brief mitochondrial permeability transition pore (mPTP) openings play an important physiological role maintaining healthy mitochondria homeostasis. Adaptive and maladaptive responses to redox stress may involve mitochondrial channels such as mPTP and inner membrane anion channel (IMAC). Their activation causes intra- and intermitochondrial redox-environment changes leading to ROS release. This regenerative cycle of mitochondrial ROS formation and release was named ROS-induced ROS release (RIRR). Brief, reversible mPTP opening-associated ROS release apparently constitutes an adaptive housekeeping function by the timely release from mitochondria of accumulated potentially toxic levels of ROS (and Ca2+). At higher ROS levels, longer mPTP openings may release a ROS burst leading to destruction of mitochondria, and if propagated from mitochondrion to mitochondrion, of the cell itself. The destructive function of RIRR may serve a physiological role by removal of unwanted cells or damaged mitochondria, or cause the pathological elimination of vital and essential mitochondria and cells. The adaptive release of sufficient ROS into the vicinity of mitochondria may also activate local pools of redox-sensitive enzymes involved in protective signaling pathways that limit ischemic damage to mitochondria and cells in that area. Maladaptive mPTP- or IMAC-related RIRR may also be playing a role in aging. Because the mechanism of mitochondrial RIRR highlights the central role of mitochondria-formed ROS, we discuss all of the known ROS-producing sites (shown in vitro) and their relevance to the mitochondrial ROS production in vivo.

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Armstrong, J. S. "Mitochondrial Medicine: Pharmacological targeting of mitochondria in disease." British Journal of Pharmacology 151, no. 8 (August 2007): 1154–65. http://dx.doi.org/10.1038/sj.bjp.0707288.

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Hamilton, James, Tatiana Brustovetsky, Rajesh Khanna, and Nickolay Brustovetsky. "Mutant huntingtin does not cross the mitochondrial outer membrane." Human Molecular Genetics 29, no. 17 (August 21, 2020): 2962–75. http://dx.doi.org/10.1093/hmg/ddaa185.

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Abstract Mutant huntingtin (mHTT) is associated with mitochondria, but the exact mitochondrial location of mHTT has not been definitively established. Recently, it was reported that mHTT is present in the intermembrane space and inhibits mitochondrial protein import by interacting with TIM23, a major component of mitochondrial protein import machinery, but evidence for functional ramifications were not provided. We assessed mHTT location using synaptic and nonsynaptic mitochondria isolated from brains of YAC128 mice and subjected to alkali treatment or limited trypsin digestion. Mitochondria were purified either with discontinuous Percoll gradient or with anti-TOM22-conjugated iron microbeads. We also used mitochondria isolated from postmortem brain tissues of unaffected individuals and HD patients. Our results demonstrate that mHTT is located on the cytosolic side of the mitochondrial outer membrane (MOM) but does not cross it. This refutes the hypothesis that mHTT may interact with TIM23 and inhibit mitochondrial protein import. The levels of expression of nuclear-encoded, TIM23-transported mitochondrial proteins ACO2, TUFM, IDH3A, CLPP and mitochondrially encoded and synthesized protein mtCO1 were similar in mitochondria from YAC128 mice and their wild-type littermates as well as in mitochondria from postmortem brain tissues of unaffected individuals and HD patients, supporting the lack of deficit in mitochondrial protein import. Regardless of purification technique, mitochondria from YAC128 and WT mice had similar respiratory activities and mitochondrial membrane potentials. Thus, our data argue against mHTT crossing the MOM and entering into the mitochondrial intermembrane space, making it highly unlikely that mHTT interacts with TIM23 and inhibits protein import in intact mitochondria.

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Lucini, Chantal B., and Ralf J. Braun. "Mitochondrion-Dependent Cell Death in TDP-43 Proteinopathies." Biomedicines 9, no. 4 (April 2, 2021): 376. http://dx.doi.org/10.3390/biomedicines9040376.

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In the last decade, pieces of evidence for TDP-43-mediated mitochondrial dysfunction in neurodegenerative diseases have accumulated. In patient samples, in vitro and in vivo models have shown mitochondrial accumulation of TDP-43, concomitantly with hallmarks of mitochondrial destabilization, such as increased production of reactive oxygen species (ROS), reduced level of oxidative phosphorylation (OXPHOS), and mitochondrial membrane permeabilization. Incidences of TDP-43-dependent cell death, which depends on mitochondrial DNA (mtDNA) content, is increased upon ageing. However, the molecular pathways behind mitochondrion-dependent cell death in TDP-43 proteinopathies remained unclear. In this review, we discuss the role of TDP-43 in mitochondria, as well as in mitochondrion-dependent cell death. This review includes the recent discovery of the TDP-43-dependent activation of the innate immunity cyclic GMP-AMP synthase/stimulator of interferon genes (cGAS/STING) pathway. Unravelling cell death mechanisms upon TDP-43 accumulation in mitochondria may open up new opportunities in TDP-43 proteinopathy research.

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Dong, Lanfeng, Vinod Gopalan, Olivia Holland, and Jiri Neuzil. "Mitocans Revisited: Mitochondrial Targeting as Efficient Anti-Cancer Therapy." International Journal of Molecular Sciences 21, no. 21 (October 26, 2020): 7941. http://dx.doi.org/10.3390/ijms21217941.

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Mitochondria are essential cellular organelles, controlling multiple signalling pathways critical for cell survival and cell death. Increasing evidence suggests that mitochondrial metabolism and functions are indispensable in tumorigenesis and cancer progression, rendering mitochondria and mitochondrial functions as plausible targets for anti-cancer therapeutics. In this review, we summarised the major strategies of selective targeting of mitochondria and their functions to combat cancer, including targeting mitochondrial metabolism, the electron transport chain and tricarboxylic acid cycle, mitochondrial redox signalling pathways, and ROS homeostasis. We highlight that delivering anti-cancer drugs into mitochondria exhibits enormous potential for future cancer therapeutic strategies, with a great advantage of potentially overcoming drug resistance. Mitocans, exemplified by mitochondrially targeted vitamin E succinate and tamoxifen (MitoTam), selectively target cancer cell mitochondria and efficiently kill multiple types of cancer cells by disrupting mitochondrial function, with MitoTam currently undergoing a clinical trial.

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Shen, Liang, and Xianquan Zhan. "Mitochondrial Dysfunction Pathway Alterations Offer Potential Biomarkers and Therapeutic Targets for Ovarian Cancer." Oxidative Medicine and Cellular Longevity 2022 (April 20, 2022): 1–22. http://dx.doi.org/10.1155/2022/5634724.

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The mitochondrion is a very versatile organelle that participates in some important cancer-associated biological processes, including energy metabolism, oxidative stress, mitochondrial DNA (mtDNA) mutation, cell apoptosis, mitochondria-nuclear communication, dynamics, autophagy, calcium overload, immunity, and drug resistance in ovarian cancer. Multiomics studies have found that mitochondrial dysfunction, oxidative stress, and apoptosis signaling pathways act in human ovarian cancer, which demonstrates that mitochondria play critical roles in ovarian cancer. Many molecular targeted drugs have been developed against mitochondrial dysfunction pathways in ovarian cancer, including olive leaf extract, nilotinib, salinomycin, Sambucus nigra agglutinin, tigecycline, and eupatilin. This review article focuses on the underlying biological roles of mitochondrial dysfunction in ovarian cancer progression based on omics data, potential molecular relationship between mitochondrial dysfunction and oxidative stress, and future perspectives of promising biomarkers and therapeutic targets based on the mitochondrial dysfunction pathway for ovarian cancer.

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Kokkinopoulou, Ioanna, and Paraskevi Moutsatsou. "Mitochondrial Glucocorticoid Receptors and Their Actions." International Journal of Molecular Sciences 22, no. 11 (June 3, 2021): 6054. http://dx.doi.org/10.3390/ijms22116054.

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Mitochondria are membrane organelles present in almost all eukaryotic cells. In addition to their well-known role in energy production, mitochondria regulate central cellular processes, including calcium homeostasis, Reactive Oxygen Species (ROS) generation, cell death, thermogenesis, and biosynthesis of lipids, nucleic acids, and steroid hormones. Glucocorticoids (GCs) regulate the mitochondrially encoded oxidative phosphorylation gene expression and mitochondrial energy metabolism. The identification of Glucocorticoid Response Elements (GREs) in mitochondrial sequences and the detection of Glucocorticoid Receptor (GR) in mitochondria of different cell types gave support to hypothesis that mitochondrial GR directly regulates mitochondrial gene expression. Numerous studies have revealed changes in mitochondrial gene expression alongside with GR import/export in mitochondria, confirming the direct effects of GCs on mitochondrial genome. Further evidence has made clear that mitochondrial GR is involved in mitochondrial function and apoptosis-mediated processes, through interacting or altering the distribution of Bcl2 family members. Even though its exact translocation mechanisms remain unknown, data have shown that GR chaperones (Hsp70/90, Bag-1, FKBP51), the anti-apoptotic protein Bcl-2, the HDAC6- mediated deacetylation and the outer mitochondrial translocation complexes (Tom complexes) co-ordinate GR mitochondrial trafficking. A role of mitochondrial GR in stress and depression as well as in lung and hepatic inflammation has also been demonstrated.

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Ding, Qianqian, Yanxiang Qi, and Suk-Ying Tsang. "Mitochondrial Biogenesis, Mitochondrial Dynamics, and Mitophagy in the Maturation of Cardiomyocytes." Cells 10, no. 9 (September 18, 2021): 2463. http://dx.doi.org/10.3390/cells10092463.

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Pluripotent stem cells (PSCs) can undergo unlimited self-renewal and can differentiate into all the cell types present in our body, including cardiomyocytes. Therefore, PSCs can be an excellent source of cardiomyocytes for future regenerative medicine and medical research studies. However, cardiomyocytes obtained from PSC differentiation culture are regarded as immature structurally, electrophysiologically, metabolically, and functionally. Mitochondria are organelles responsible for various cellular functions such as energy metabolism, different catabolic and anabolic processes, calcium fluxes, and various signaling pathways. Cells can respond to cellular needs to increase the mitochondrial mass by mitochondrial biogenesis. On the other hand, cells can also degrade mitochondria through mitophagy. Mitochondria are also dynamic organelles that undergo continuous fusion and fission events. In this review, we aim to summarize previous findings on the changes of mitochondrial biogenesis, mitophagy, and mitochondrial dynamics during the maturation of cardiomyocytes. In addition, we intend to summarize whether changes in these processes would affect the maturation of cardiomyocytes. Lastly, we aim to discuss unanswered questions in the field and to provide insights for the possible strategies of enhancing the maturation of PSC-derived cardiomyocytes.

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Knorre, Dmitry A., Konstantin Y. Popadin, Svyatoslav S. Sokolov, and Fedor F. Severin. "Roles of Mitochondrial Dynamics under Stressful and Normal Conditions in Yeast Cells." Oxidative Medicine and Cellular Longevity 2013 (2013): 1–6. http://dx.doi.org/10.1155/2013/139491.

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Eukaryotic cells contain dynamic mitochondrial filaments: they fuse and divide. Here we summarize data on the protein machinery driving mitochondrial dynamics in yeast and also discuss the factors that affect the fusion-fission balance. Fission is a general stress response of cells, and in the case of yeast this response appears to be prosurvival. At the same time, even under normal conditions yeast mitochondria undergo continuous cycles of fusion and fission. This seems to be a futile cycle and also expensive from the energy point of view. Why does it exist? Benefits might be the same as in the case of sexual reproduction. Indeed, mixing and separating of mitochondrial content allows mitochondrial DNA to segregate and recombine randomly, leading to high variation in the numbers of mutations per individual mitochondrion. This opens a possibility for effective purifying selection-elimination of mitochondria highly contaminated by deleterious mutations. The beneficial action presumes a mechanism for removal of defective mitochondria. We argue that selective mitochondrial autophagy or asymmetrical distribution of mitochondria during cell division could be at the core of such mechanism.

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ABE, Takaaki. "1. Mitochondrial Medicine." Rinsho yakuri/Japanese Journal of Clinical Pharmacology and Therapeutics 48, no. 3 (2017): 106–9. http://dx.doi.org/10.3999/jscpt.48.106.

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Murri, Luigi. "Foreword: Mitochondrial Medicine." Bioscience Reports 27, no. 1-3 (June 13, 2007): 1–3. http://dx.doi.org/10.1007/s10540-007-9031-6.

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Mitochondrion is currently known to play major roles in many disease processes: neuromuscular disorders, neurodegenerative conditions (i.e. Parkinson's and Alzheimer's diseases), diabetes mellitus, aging, programmed cell death, and carcinogenesis, to name a few. In this background, the Department of Neuroscience of the University of Pisa (Italy) has organised a scientific meeting on October 25th, 2006, to discuss recent progress in the field of mitochondriology.

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DiMauro, Salvatore. "Mitochondrial DNA Medicine." Bioscience Reports 27, no. 1-3 (June 13, 2007): 5–9. http://dx.doi.org/10.1007/s10540-007-9032-5.

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The small, maternally inherited mitochondrial DNA (mtDNA) has turned out to be a hotbed of pathogenic mutations: 15 years into the era of ‘mitochondrial medicine’, over 150 pathogenic point mutations and countless rearrangements have been associated with a variety of multisystemic or tissue-specific human diseases. MtDNA-related disorders can be divided into two major groups: those due to mutations in genes affecting mitochondrial protein synthesis in toto and those due to mutations in specific protein-coding genes. Here we review the mitochondrial genetics and the clinical features of the mtDNA-related diseases.

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Wallace, Douglas C. "Mitochondrial genetic medicine." Nature Genetics 50, no. 12 (October 29, 2018): 1642–49. http://dx.doi.org/10.1038/s41588-018-0264-z.

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Demaerschalk, Bart M. "Clinical Mitochondrial Medicine." Neurologist 23, no. 6 (November 2018): 209. http://dx.doi.org/10.1097/nrl.0000000000000216.

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Zeviani, Massimo, and Carlo Viscomi. "Mitochondrial Neurodegeneration." Cells 11, no. 4 (February 11, 2022): 637. http://dx.doi.org/10.3390/cells11040637.

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Mitochondria are cytoplasmic organelles, which generate energy as heat and ATP, the universal energy currency of the cell. This process is carried out by coupling electron stripping through oxidation of nutrient substrates with the formation of a proton-based electrochemical gradient across the inner mitochondrial membrane. Controlled dissipation of the gradient can lead to production of heat as well as ATP, via ADP phosphorylation. This process is known as oxidative phosphorylation, and is carried out by four multiheteromeric complexes (from I to IV) of the mitochondrial respiratory chain, carrying out the electron flow whose energy is stored as a proton-based electrochemical gradient. This gradient sustains a second reaction, operated by the mitochondrial ATP synthase, or complex V, which condensates ADP and Pi into ATP. Four complexes (CI, CIII, CIV, and CV) are composed of proteins encoded by genes present in two separate compartments: the nuclear genome and a small circular DNA found in mitochondria themselves, and are termed mitochondrial DNA (mtDNA). Mutations striking either genome can lead to mitochondrial impairment, determining infantile, childhood or adult neurodegeneration. Mitochondrial disorders are complex neurological syndromes, and are often part of a multisystem disorder. In this paper, we divide the diseases into those caused by mtDNA defects and those that are due to mutations involving nuclear genes; from a clinical point of view, we discuss pediatric disorders in comparison to juvenile or adult-onset conditions. The complementary genetic contributions controlling organellar function and the complexity of the biochemical pathways present in the mitochondria justify the extreme genetic and phenotypic heterogeneity of this new area of inborn errors of metabolism known as ‘mitochondrial medicine’.

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Mtango, N. R., A. J. Harvey, K. E. Latham, and C. A. Brenner. "Molecular control of mitochondrial function in developing rhesus monkey oocytes and preimplantation-stage embryos." Reproduction, Fertility and Development 20, no. 7 (2008): 846. http://dx.doi.org/10.1071/rd08078.

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The mitochondrion undergoes significant functional and structural changes, as well as an increase in number, during preimplantation embryonic development. The mitochondrion generates ATP and regulates a range of cellular processes, such as signal transduction and apoptosis. Therefore, mitochondria contribute to overall oocyte quality and embryo developmental competence. The present study identified, for the first time, the detailed temporal expression of mRNAs related to mitochondrial biogenesis in rhesus monkey oocytes and embryos. Persistent expression of maternally encoded mRNAs was observed, in combination with transcriptional activation and mRNA accumulation at the eight-cell stage, around the time of embryonic genome activation. The expression of these transcripts was significantly altered in oocytes and embryos with reduced developmental potential. In these embryos, most maternally encoded transcripts were precociously depleted. Embryo culture and specific culture media affected the expression of some of these transcripts, including a deficiency in the expression of key transcriptional regulators. Several genes involved in regulating mitochondrial transcription and replication are similarly affected by in vitro conditions and their downregulation may be instrumental in maintaining the mRNA profiles of mitochondrially encoded genes observed in the present study. These data support the hypothesis that the molecular control of mitochondrial biogenesis, and therefore mitochondrial function, is impaired in in vitro-cultured embryos. These results highlight the need for additional studies in human and non-human primate model species to determine how mitochondrial biogenesis can be altered by oocyte and embryo manipulation protocols and whether this affects physiological function in progeny.

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Sainero-Alcolado, Lourdes, Judit Liaño-Pons, María Victoria Ruiz-Pérez, and Marie Arsenian-Henriksson. "Targeting mitochondrial metabolism for precision medicine in cancer." Cell Death & Differentiation 29, no. 7 (July 2022): 1304–17. http://dx.doi.org/10.1038/s41418-022-01022-y.

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AbstractDuring decades, the research field of cancer metabolism was based on the Warburg effect, described almost one century ago. Lately, the key role of mitochondria in cancer development has been demonstrated. Many mitochondrial pathways including oxidative phosphorylation, fatty acid, glutamine, and one carbon metabolism are altered in tumors, due to mutations in oncogenes and tumor suppressor genes, as well as in metabolic enzymes. This results in metabolic reprogramming that sustains rapid cell proliferation and can lead to an increase in reactive oxygen species used by cancer cells to maintain pro-tumorigenic signaling pathways while avoiding cellular death. The knowledge acquired on the importance of mitochondrial cancer metabolism is now being translated into clinical practice. Detailed genomic, transcriptomic, and metabolomic analysis of tumors are necessary to develop more precise treatments. The successful use of drugs targeting metabolic mitochondrial enzymes has highlighted the potential for their use in precision medicine and many therapeutic candidates are in clinical trials. However, development of efficient personalized drugs has proved challenging and the combination with other strategies such as chemocytotoxic drugs, immunotherapy, and ketogenic or calorie restriction diets is likely necessary to boost their potential. In this review, we summarize the main mitochondrial features, metabolic pathways, and their alterations in different cancer types. We also present an overview of current inhibitors, highlight enzymes that are attractive targets, and discuss challenges with translation of these approaches into clinical practice. The role of mitochondria in cancer is indisputable and presents several attractive targets for both tailored and personalized cancer therapy.

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Habbane, Mouna, Julio Montoya, Taha Rhouda, Yousra Sbaoui, Driss Radallah, and Sonia Emperador. "Human Mitochondrial DNA: Particularities and Diseases." Biomedicines 9, no. 10 (October 1, 2021): 1364. http://dx.doi.org/10.3390/biomedicines9101364.

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Mitochondria are the cell’s power site, transforming energy into a form that the cell can employ for necessary metabolic reactions. These organelles present their own DNA. Although it codes for a small number of genes, mutations in mtDNA are common. Molecular genetics diagnosis allows the analysis of DNA in several areas such as infectiology, oncology, human genetics and personalized medicine. Knowing that the mitochondrial DNA is subject to several mutations which have a direct impact on the metabolism of the mitochondrion leading to many diseases, it is therefore necessary to detect these mutations in the patients involved. To date numerous mitochondrial mutations have been described in humans, permitting confirmation of clinical diagnosis, in addition to a better management of the patients. Therefore, different techniques are employed to study the presence or absence of mitochondrial mutations. However, new mutations are discovered, and to determine if they are the cause of disease, different functional mitochondrial studies are undertaken using transmitochondrial cybrid cells that are constructed by fusion of platelets of the patient that presents the mutation, with rho osteosarcoma cell line. Moreover, the contribution of next generation sequencing allows sequencing of the entire human genome within a single day and should be considered in the diagnosis of mitochondrial mutations.

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Podolak, Amira, Izabela Woclawek-Potocka, and Krzysztof Lukaszuk. "The Role of Mitochondria in Human Fertility and Early Embryo Development: What Can We Learn for Clinical Application of Assessing and Improving Mitochondrial DNA?" Cells 11, no. 5 (February 24, 2022): 797. http://dx.doi.org/10.3390/cells11050797.

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Mitochondria are well known as ‘the powerhouses of the cell’. Indeed, their major role is cellular energy production driven by both mitochondrial and nuclear DNA. Such a feature makes these organelles essential for successful fertilisation and proper embryo implantation and development. Generally, mitochondrial DNA is exclusively maternally inherited; oocyte’s mitochondrial DNA level is crucial to provide sufficient ATP content for the developing embryo until the blastocyst stage of development. Additionally, human fertility and early embryogenesis may be affected by either point mutations or deletions in mitochondrial DNA. It was suggested that their accumulation may be associated with ovarian ageing. If so, is mitochondrial dysfunction the cause or consequence of ovarian ageing? Moreover, such an obvious relationship of mitochondria and mitochondrial genome with human fertility and early embryo development gives the field of mitochondrial research a great potential to be of use in clinical application. However, even now, the area of assessing and improving DNA quantity and function in reproductive medicine drives many questions and uncertainties. This review summarises the role of mitochondria and mitochondrial DNA in human reproduction and gives an insight into the utility of their clinical use.

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Luna-Sánchez, Marta, Patrizia Bianchi, and Albert Quintana. "Mitochondria-Induced Immune Response as a Trigger for Neurodegeneration: A Pathogen from Within." International Journal of Molecular Sciences 22, no. 16 (August 7, 2021): 8523. http://dx.doi.org/10.3390/ijms22168523.

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Symbiosis between the mitochondrion and the ancestor of the eukaryotic cell allowed cellular complexity and supported life. Mitochondria have specialized in many key functions ensuring cell homeostasis and survival. Thus, proper communication between mitochondria and cell nucleus is paramount for cellular health. However, due to their archaebacterial origin, mitochondria possess a high immunogenic potential. Indeed, mitochondria have been identified as an intracellular source of molecules that can elicit cellular responses to pathogens. Compromised mitochondrial integrity leads to release of mitochondrial content into the cytosol, which triggers an unwanted cellular immune response. Mitochondrial nucleic acids (mtDNA and mtRNA) can interact with the same cytoplasmic sensors that are specialized in recognizing genetic material from pathogens. High-energy demanding cells, such as neurons, are highly affected by deficits in mitochondrial function. Notably, mitochondrial dysfunction, neurodegeneration, and chronic inflammation are concurrent events in many severe debilitating disorders. Interestingly in this context of pathology, increasing number of studies have detected immune-activating mtDNA and mtRNA that induce an aberrant production of pro-inflammatory cytokines and interferon effectors. Thus, this review provides new insights on mitochondria-driven inflammation as a potential therapeutic target for neurodegenerative and primary mitochondrial diseases.

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Murphy, Michael P., and Robin A. J. Smith. "Drug delivery to mitochondria: the key to mitochondrial medicine." Advanced Drug Delivery Reviews 41, no. 2 (March 2000): 235–50. http://dx.doi.org/10.1016/s0169-409x(99)00069-1.

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Garipi, Enis, Aleksandra Rakovac, Otto Barak, Damir Lukac, Nada Naumovic, Miodrag Drapsin, Dea Karaba, et al. "In situ analysis of mitochondrial respiratory capacity - foundation for cellular physiology." Medical review 70, no. 11-12 (2017): 445–48. http://dx.doi.org/10.2298/mpns1712445g.

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Mitochondria are ubiquitous organelles of eukaryotic cells and they are the mayor site of generating energy in the form of adenosine triphoshate through the process of oxidative phosphorylation. Analysis and estimation of mitochondrial function is of outmost importance when it comes to studying intracellular energy metabolism, mechanisms of apoptosis, signaling pathways, calcium storage and the pathophysiology of a large spectrum of human diseases including various neurodegenerative diseases, myopathies, metabolic syndromes and cancer. Respiratory capacity analysis covers one of the many roles that mitochondria play in living cells and it provides us with useful data about functional integrity of mitochondria. Assessment of individual respiratory chain complexes or other mitochondrial enzymes has been widely used to estimate mitochondrial function and dysfunction but it neglects the influence of complex structural and functional interplay of mitochondrial proteins and enzymes and plasmic compounds. Another method that emphasises the importance of studying intact mitochondria is in vitro technique, and although it has many advantages, in some aspects it is far from being representative when it comes to functional assessment of mitochondria. From the perspective of energy production and consumption, the cardiac muscle is a highly demanding tissue and it is the well functioning of mitochondria that is conditio sine qua non for this nature to be fulfilled. In cooperation with the University of Split School of Medicine in Split and under the mentorship of Prof. Marko Ljubkovic, the Department of Physiology of the Faculty of Medicine Novi Sad works on introducing in situ approaches in the analysis of respiratory mitochondrial function in skinned muscle fibers of human cardiac tissue.

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Frye, Richard E., Janet Cakir, Shannon Rose, Raymond F. Palmer, Christine Austin, Paul Curtin, and Manish Arora. "Mitochondria May Mediate Prenatal Environmental Influences in Autism Spectrum Disorder." Journal of Personalized Medicine 11, no. 3 (March 18, 2021): 218. http://dx.doi.org/10.3390/jpm11030218.

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We propose that the mitochondrion, an essential cellular organelle, mediates the long-term prenatal environmental effects of disease in autism spectrum disorder (ASD). Many prenatal environmental factors which increase the risk of developing ASD influence mitochondria physiology, including toxicant exposures, immune activation, and nutritional factors. Unique types of mitochondrial dysfunction have been associated with ASD and recent studies have linked prenatal environmental exposures to long-term changes in mitochondrial physiology in children with ASD. A better understanding of the role of the mitochondria in the etiology of ASD can lead to targeted therapeutics and strategies to potentially prevent the development of ASD.

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Frantsiyants, E. M., I. V. Neskubina, and E. A. Sheiko. "Mitochondria of transformed cell as a target of antitumor influence." Research and Practical Medicine Journal 7, no. 2 (June 25, 2020): 92–108. http://dx.doi.org/10.17709/2409-2231-2020-7-2-9.

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Mitochondria are intracellular organelles in eukaryotic cells that participate in bioenergy metabolism and cell homeostasis, including ATP generation through electron transport and oxidative phosphorylation in combination with oxidation of metabolites by the tricarboxylic acid cycle and fatty acid catabolism via β-oxidation. the production of reactive oxygen species, as well as the initiation and implementation of apoptosis. Mitochondria play a crucial role in cellular energy metabolism and the regulation of programmed cell death. mitochondria activate numerous signaling pathways associated with cell death. Mitochondria have the ability to control the activation of programmed cell death by regulating the translocation of proapoptotic proteins from the intermediate space of mitochondria to the cytosol. This is the reason for the emergence of a new discipline — mitochondrial medicine. The review examined and analyzed scientific publications on the role of mitochondria in the life support of transformed cells, the study of their functioning and structurally functional dysfunctions, as part of mitochondrial medicine. Mitochondrial medicine is a developing discipline whose significance stems from the central function of mitochondria in the production of adenosine triphosphate, the generation of reactive oxygen species, and cell death due to necrosis or apoptosis. Consequently, mitochondrial dysfunction plays an important role in the pathophysiology of cancer, many other common diseases and side effects of drugs. Perhaps the combined use of modulators of mitochondrial metabolism and antitumor therapy will contribute to the emergence of a new direction in antitumor treatment, which will significantly increase the effectiveness of cancer treatment.

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Włoga, D., I. Strzyżewska-Jówko, J. Gaertig, and M. Jerka-Dziadosz. "Septins Stabilize Mitochondria in Tetrahymena thermophila." Eukaryotic Cell 7, no. 8 (June 27, 2008): 1373–86. http://dx.doi.org/10.1128/ec.00085-08.

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ABSTRACT We describe phylogenetic and functional studies of three septins in the free-living ciliate Tetrahymena thermophila. Both deletion and overproduction of septins led to vacuolization of mitochondria, destabilization of the nuclear envelope, and increased autophagy. All three green fluorescent protein-tagged septins localized to mitochondria. Specific septins localized to the outer mitochondrial membrane, to septa formed during mitochondrial scission, or to the mitochondrion-associated endoplasmic reticulum. The only other septins known to localize to mitochondria are human ARTS and murine M-septin, both alternatively spliced forms of Sep4 (S. Larisch, Cell Cycle 3:1021-1023, 2004; S. Takahashi, R. Inatome, H. Yamamura, and S. Yanagi, Genes Cells 8:81-93, 2003). It therefore appears that septins have been recruited to mitochondrial functions independently in at least two eukaryotic lineages and in both cases are involved in apoptotic events.

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Yamada, Yuma, Momo Ito, Manae Arai, Mitsue Hibino, Takao Tsujioka, and Hideyoshi Harashima. "Challenges in Promoting Mitochondrial Transplantation Therapy." International Journal of Molecular Sciences 21, no. 17 (September 2, 2020): 6365. http://dx.doi.org/10.3390/ijms21176365.

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Mitochondrial transplantation therapy is an innovative strategy for the treatment of mitochondrial dysfunction. The approach has been reported to be useful in the treatment of cardiac ischemic reperfusion injuries in human clinical trials and has also been shown to be useful in animal studies as a method for treating mitochondrial dysfunction in various tissues, including the heart, liver, lungs, and brain. On the other hand, there is no methodology for using preserved mitochondria. Research into the pharmaceutical formulation of mitochondria to promote mitochondrial transplantation therapy as the next step in treating many patients is urgently needed. In this review, we overview previous studies on the therapeutic effects of mitochondrial transplantation. We also discuss studies related to immune responses that occur during mitochondrial transplantation and methods for preserving mitochondria, which are key to their stability as medicines. Finally, we describe research related to mitochondrial targeting drug delivery systems (DDS) and discuss future perspectives of mitochondrial transplantation.

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Ozawa, T. "Genetic and functional changes in mitochondria associated with aging." Physiological Reviews 77, no. 2 (April 1, 1997): 425–64. http://dx.doi.org/10.1152/physrev.1997.77.2.425.

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This review is devoted to the molecular genetics and bioenergetics of human mitochondria related to the mechanism of aging. Morphological and functional changes of mitochondria associated with age and age-related disease are overviewed with special reference to the changes in enzymes encoded by mitochondrial-inherent genome. The somatically acquired mutations and oxidative damage of the genome, which lead an individual to the fragmentation of mitochondrial DNA, cellular energy crisis, naturally occurring cell death (apoptosis), and tissue degeneration and atrophy, are reviewed with relation to the inherited point mutational genotypes and the deletion types of mitochondrial DNA. Theories of aging are discussed with disclosed evidence relevant to them. Some trials to prevent age-related damage in mitochondria are introduced for the development of what may be called mitochondrial medicine.

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Haseeb, Abdul, Hong Chen, Yufei Huang, Ping Yang, Xuejing Sun, Adeela Iqbal, Nisar Ahmed, et al. "Remodelling of mitochondria during spermiogenesis of Chinese soft-shelled turtle (Pelodiscus sinensis)." Reproduction, Fertility and Development 30, no. 11 (2018): 1514. http://dx.doi.org/10.1071/rd18010.

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Mitochondria are vital cellular organelles that have the ability to change their shape under different conditions, such as in response to stress, disease, changes in metabolic rate, energy requirements and apoptosis. In the present study, we observed remodelling of mitochondria during spermiogenesis and its relationship with mitochondria-associated granules (MAG). At the beginning of spermiogenesis, mitochondria are characterised by their round shape. As spermiogenesis progresses, the round-shaped mitochondria change into elongated and then swollen mitochondria, subsequently forming a crescent-like shape and finally developing into onion-like shaped mitochondria. We also noted changes in mitochondrial size, location and patterns of cristae at different stages of spermiogenesis. Significant differences (P < 0.0001) were found in the size of the different-shaped mitochondria. In early spermatids transitioning to the granular nucleus stage, the size of the mitochondria decreased, but increased subsequently during spermiogenesis. Changes in size and morphological variations were achieved through marked mitochondrial fusion. We also observed a non-membranous structure (MAG) closely associated with mitochondria that may stimulate or control fusion during mitochondrial remodelling. The end product of this sophisticated remodelling process in turtle spermatozoa is an onion-like mitochondrion. The acquisition of this kind of mitochondrial configuration is one strategy for long-term sperm storage in turtles.

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Ienco, Elena Caldarazzo, Costanza Simoncini, Daniele Orsucci, Loredana Petrucci, Massimiliano Filosto, Michelangelo Mancuso, and Gabriele Siciliano. "May “Mitochondrial Eve” and Mitochondrial Haplogroups Play a Role in Neurodegeneration and Alzheimer's Disease?" International Journal of Alzheimer's Disease 2011 (2011): 1–11. http://dx.doi.org/10.4061/2011/709061.

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Mitochondria, the powerhouse of the cell, play a critical role in several metabolic processes and apoptotic pathways. Multiple evidences suggest that mitochondria may be crucial in ageing-related neurodegenerative diseases. Moreover, mitochondrial haplogroups have been linked to multiple area of medicine, from normal ageing to diseases, including neurodegeneration. Polymorphisms within the mitochondrial genome might lead to impaired energy generation and to increased amount of reactive oxygen species, having either susceptibility or protective role in several diseases. Here, we highlight the role of the mitochondrial haplogroups in the pathogenetic cascade leading to diseases, with special attention to Alzheimer's disease.

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Valdés-Aguayo, José J., Idalia Garza-Veloz, José I. Badillo-Almaráz, Sofia Bernal-Silva, Maria C. Martínez-Vázquez, Vladimir Juárez-Alcalá, José R. Vargas-Rodríguez, et al. "Mitochondria and Mitochondrial DNA: Key Elements in the Pathogenesis and Exacerbation of the Inflammatory State Caused by COVID-19." Medicina 57, no. 9 (September 3, 2021): 928. http://dx.doi.org/10.3390/medicina57090928.

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Background and Objectives. The importance of mitochondria in inflammatory pathologies, besides providing energy, is associated with the release of mitochondrial damage products, such as mitochondrial DNA (mt-DNA), which may perpetuate inflammation. In this review, we aimed to show the importance of mitochondria, as organelles that produce energy and intervene in multiple pathologies, focusing mainly in COVID-19 and using multiple molecular mechanisms that allow for the replication and maintenance of the viral genome, leading to the exacerbation and spread of the inflammatory response. The evidence suggests that mitochondria are implicated in the replication of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), which forms double-membrane vesicles and evades detection by the cell defense system. These mitochondrion-hijacking vesicles damage the integrity of the mitochondrion’s membrane, releasing mt-DNA into circulation and triggering the activation of innate immunity, which may contribute to an exacerbation of the pro-inflammatory state. Conclusions. While mitochondrial dysfunction in COVID-19 continues to be studied, the use of mt-DNA as an indicator of prognosis and severity is a potential area yet to be explored.

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Hollander, John M., Dharendra Thapa, and Danielle L. Shepherd. "Physiological and structural differences in spatially distinct subpopulations of cardiac mitochondria: influence of cardiac pathologies." American Journal of Physiology-Heart and Circulatory Physiology 307, no. 1 (July 1, 2014): H1—H14. http://dx.doi.org/10.1152/ajpheart.00747.2013.

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Cardiac tissue contains discrete pools of mitochondria that are characterized by their subcellular spatial arrangement. Subsarcolemmal mitochondria (SSM) exist below the cell membrane, interfibrillar mitochondria (IFM) reside in rows between the myofibrils, and perinuclear mitochondria are situated at the nuclear poles. Microstructural imaging of heart tissue coupled with the development of differential isolation techniques designed to sequentially separate spatially distinct mitochondrial subpopulations have revealed differences in morphological features including shape, absolute size, and internal cristae arrangement. These findings have been complemented by functional studies indicating differences in biochemical parameters and, potentially, functional roles for the ATP generated, based upon subcellular location. Consequently, mitochondrial subpopulations appear to be influenced differently during cardiac pathologies including ischemia/reperfusion, heart failure, aging, exercise, and diabetes mellitus. These influences may be the result of specific structural and functional disparities between mitochondrial subpopulations such that the stress elicited by a given cardiac insult differentially impacts subcellular locales and the mitochondria contained within. The goal of this review is to highlight some of the inherent structural and functional differences that exist between spatially distinct cardiac mitochondrial subpopulations as well as provide an overview of the differential impact of various cardiac pathologies on spatially distinct mitochondrial subpopulations. As an outcome, we will instill a basis for incorporating subcellular spatial location when evaluating the impact of cardiac pathologies on the mitochondrion. Incorporation of subcellular spatial location may offer the greatest potential for delineating the influence of cardiac pathology on this critical organelle.

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Sonn, Seong Keun, Seungwoon Seo, Jaemoon Yang, Ki Sook Oh, Hsiuchen Chen, David C. Chan, Kunsoo Rhee, Kyung S. Lee, Young Yang, and Goo Taeg Oh. "ER-associated CTRP1 regulates mitochondrial fission via interaction with DRP1." Experimental & Molecular Medicine 53, no. 11 (November 2021): 1769–80. http://dx.doi.org/10.1038/s12276-021-00701-z.

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AbstractC1q/TNF-related protein 1 (CTRP1) is a CTRP family member that has collagenous and globular C1q-like domains. The secreted form of CTRP1 is known to be associated with cardiovascular and metabolic diseases, but its cellular roles have not yet been elucidated. Here, we showed that cytosolic CTRP1 localizes to the endoplasmic reticulum (ER) membrane and that knockout or depletion of CTRP1 leads to mitochondrial fission defects, as demonstrated by mitochondrial elongation. Mitochondrial fission events are known to occur through an interaction between mitochondria and the ER, but we do not know whether the ER and/or its associated proteins participate directly in the entire mitochondrial fission event. Interestingly, we herein showed that ablation of CTRP1 suppresses the recruitment of DRP1 to mitochondria and provided evidence suggesting that the ER–mitochondrion interaction is required for the proper regulation of mitochondrial morphology. We further report that CTRP1 inactivation-induced mitochondrial fission defects induce apoptotic resistance and neuronal degeneration, which are also associated with ablation of DRP1. These results demonstrate for the first time that cytosolic CTRP1 is an ER transmembrane protein that acts as a key regulator of mitochondrial fission, providing new insight into the etiology of metabolic and neurodegenerative disorders.

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Rishi Paliwal, Shivani Rai, Bhuvaneshwar Vaidya, Sunil Mahor, Prem N. Gupta, Amit Rawat, and S.P. Vyas. "Cell-Selective Mitochondrial Targeting: Progress in Mitochondrial Medicine." Current Drug Delivery 4, no. 3 (July 1, 2007): 211–24. http://dx.doi.org/10.2174/156720107781023910.

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Journal articles on the topic 'Mitochondrial medicine'

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