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Journal articles on the topic 'G93A-SOD1 mice'

Author: Grafiati

Published: 9 March 2023

Last updated: 10 March 2023

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1

Aishwarya, Richa, Chowdhury S. Abdullah, Naznin Sultana Remex, Sadia Nitu, Brandon Hartman, Judy King, Mohammad Alfrad Nobel Bhuiyan, et al. "Pathological Sequelae Associated with Skeletal Muscle Atrophy and Histopathology in G93A*SOD1 Mice." Muscles 2, no. 1 (February 2, 2023): 51–74. http://dx.doi.org/10.3390/muscles2010006.

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Amyotrophic lateral sclerosis (ALS) is a complex systemic disease that primarily involves motor neuron dysfunction and skeletal muscle atrophy. One commonly used mouse model to study ALS was generated by transgenic expression of a mutant form of human superoxide dismutase 1 (SOD1) gene harboring a single amino acid substitution of glycine to alanine at codon 93 (G93A*SOD1). Although mutant-SOD1 is ubiquitously expressed in G93A*SOD1 mice, a detailed analysis of the skeletal muscle expression pattern of the mutant protein and the resultant muscle pathology were never performed. Using different skeletal muscles isolated from G93A*SOD1 mice, we extensively characterized the pathological sequelae of histological, molecular, ultrastructural, and biochemical alterations. Muscle atrophy in G93A*SOD1 mice was associated with increased and differential expression of mutant-SOD1 across myofibers and increased MuRF1 protein level. In addition, high collagen deposition and myopathic changes sections accompanied the reduced muscle strength in the G93A*SOD1 mice. Furthermore, all the muscles in G93A*SOD1 mice showed altered protein levels associated with different signaling pathways, including inflammation, mitochondrial membrane transport, mitochondrial lipid uptake, and antioxidant enzymes. In addition, the mutant-SOD1 protein was found in the mitochondrial fraction in the muscles from G93A*SOD1 mice, which was accompanied by vacuolized and abnormal mitochondria, altered OXPHOS and PDH complex protein levels, and defects in mitochondrial respiration. Overall, we reported the pathological sequelae observed in the skeletal muscles of G93A*SOD1 mice resulting from the whole-body mutant-SOD1 protein expression.

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2

Apolloni, Savina, Francesca Caputi, Annabella Pignataro, Susanna Amadio, Paola Fabbrizio, Martine Ammassari-Teule, and Cinzia Volonté. "Histamine Is an Inducer of the Heat Shock Response in SOD1-G93A Models of ALS." International Journal of Molecular Sciences 20, no. 15 (August 3, 2019): 3793. http://dx.doi.org/10.3390/ijms20153793.

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(1) Background: Amyotrophic lateral sclerosis (ALS) is a multifactorial non-cell autonomous disease where activation of microglia and astrocytes largely contributes to motor neurons death. Heat shock proteins have been demonstrated to promote neuronal survival and exert a strong anti-inflammatory action in glia. Having previously shown that the pharmacological increase of the histamine content in the central nervous system (CNS) of SOD1-G93A mice decreases neuroinflammation, reduces motor neuron death, and increases mice life span, here we examined whether this effect could be mediated by an enhancement of the heat shock response. (2) Methods: Heat shock protein expression was analyzed in vitro and in vivo. Histamine was provided to primary microglia and NSC-34 motor neurons expressing the SOD1-G93A mutation. The brain permeable histamine precursor histidine was chronically administered to symptomatic SOD1-G93A mice. Spine density was measured by Golgi-staining in motor cortex of histidine-treated SOD1-G93A mice. (3) Results: We demonstrate that histamine activates the heat shock response in cultured SOD1-G93A microglia and motor neurons. In SOD1-G93A mice, histidine augments the protein content of GRP78 and Hsp70 in spinal cord and cortex, where the treatment also rescues type I motor neuron dendritic spine loss. (4) Conclusion: Besides the established histaminergic neuroprotective and anti-inflammatory effects, the induction of the heat shock response in the SOD1-G93A model by histamine confirms the importance of this pathway in the search for successful therapeutic solutions to treat ALS.

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3

Lei, Hongxia, Elisabeth Dirren, Carole Poitry-Yamate, Bernard L. Schneider, Rolf Gruetter, and Patrick Aebischer. "Evolution of the neurochemical profiles in the G93A-SOD1 mouse model of amyotrophic lateral sclerosis." Journal of Cerebral Blood Flow & Metabolism 39, no. 7 (February 5, 2018): 1283–98. http://dx.doi.org/10.1177/0271678x18756499.

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In vivo 1H magnetic resonance spectroscopy (1H-MRS) investigations of amyotrophic lateral sclerosis (ALS) mouse brain may provide neurochemical profiles and alterations in association with ALS disease progression. We aimed to longitudinally follow neurochemical evolutions of striatum, brainstem and motor cortex of mice transgenic for G93A mutant human superoxide dismutase type-1 (G93A-SOD1), an ALS model. Region-specific neurochemical alterations were detected in asymptomatic G93A-SOD1 mice, particularly in lactate (−19%) and glutamate (+8%) of brainstem, along with γ-amino-butyric acid (−30%), N-acetyl-aspartate (−5%) and ascorbate (+51%) of motor cortex. With disease progression towards the end-stage, increased numbers of metabolic changes of G93A-SOD1 mice were observed (e.g. glutamine levels increased in the brainstem (>+66%) and motor cortex (>+54%)). Through ALS disease progression, an overall increase of glutamine/glutamate in G93A-SOD1 mice was observed in the striatum ( p < 0.01) and even more so in two motor neuron enriched regions, the brainstem and motor cortex ( p < 0.0001). These 1H-MRS data underscore a pattern of neurochemical alterations that are specific to brain regions and to disease stages of the G93A-SOD1 mouse model. These neurochemical changes may contribute to early diagnosis and disease monitoring in ALS patients.

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Deng, Binbin, Wenjing Lv, Weisong Duan, Yakun Liu, Zhongyao Li, Yanqin Ma, Guisen Zhang, et al. "Progressive Degeneration and Inhibition of Peripheral Nerve Regeneration in the SOD1-G93A Mouse Model of Amyotrophic Lateral Sclerosis." Cellular Physiology and Biochemistry 46, no. 6 (2018): 2358–72. http://dx.doi.org/10.1159/000489627.

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Background: Myelination, degeneration and regeneration are implicated in crucial responses to injury in the peripheral nervous system. Considering the progression of amyotrophic lateral sclerosis (ALS), we used the superoxide dismutase 1 (SOD1)-G93A transgenic mouse model of ALS to investigate the effects of mutant SOD1 on the peripheral nerves. Methods: Changes in peripheral nerve morphology were analyzed in SOD1 mutant mice at various stages of the disease by toluidine blue staining and electron microscopy (EM). Schwann cell proliferation and recruitment of inflammatory factors were detected by immunofluorescence staining and quantitative reverse transcription PCR and were compared between SOD1 mutant mice and control mice. Furthermore, western blotting (WB) and TUNEL staining were used to investigate axonal damage and Schwann cell survival in the sciatic nerves of mice in both groups. Results: An analysis of the peripheral nervous system in SOD1-G93A mice revealed the following novel features: (i) Schwann cells and axons in mutant mice underwent changes that were similar to those seen in the control mice during the early development of peripheral nerves. (ii) The peripheral nerves of SOD1-G93A mice developed progressive neuropathy, which presented as defects in axons and myelin, leading to difficulty in walking and reduced locomotor capacity at a late stage of the disease. (iii) Macrophages were recruited and accumulated, and nerve injury and a deficit in the blood-nerve barrier were observed. (iv) Proliferation and the inflammatory micro-environment were inhibited, which impaired the regeneration and remyelination of axons after crush injury in the SOD1-G93A mice. Conclusions: The mutant human SOD1 protein induced axonal and myelin degeneration during the progression of ALS and participated in axon remyelination and regeneration in response to injury.

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5

Marcuzzo, Stefania, Davide Isaia, Silvia Bonanno, Claudia Malacarne, Paola Cavalcante, Antonella Zacheo, Valentino Laquintana, et al. "FM19G11-Loaded Gold Nanoparticles Enhance the Proliferation and Self-Renewal of Ependymal Stem Progenitor Cells Derived from ALS Mice." Cells 8, no. 3 (March 23, 2019): 279. http://dx.doi.org/10.3390/cells8030279.

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Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease affecting motor neurons. In ALS mice, neurodegeneration is associated with the proliferative restorative attempts of ependymal stem progenitor cells (epSPCs) that normally lie in a quiescent in the spinal cord. Thus, modulation of the proliferation of epSPCs may represent a potential strategy to counteract neurodegeneration. Recent studies demonstrated that FM19G11, a hypoxia-inducible factor modulator, induces epSPC self-renewal and proliferation. The aim of the study was to investigate whether FM19G11-loaded gold nanoparticles (NPs) can affect self-renewal and proliferation processes in epSPCs isolated from G93A-SOD1 mice at disease onset. We discovered elevated levels of SOX2, OCT4, AKT1, and AKT3, key genes associated with pluripotency, self-renewal, and proliferation, in G93A-SOD1 epSPCs at the transcriptional and protein levels after treatment with FM19G11-loaded NPs. We also observed an increase in the levels of the mitochondrial uncoupling protein (UCP) gene in treated cells. FM19G11-loaded NPs treatment also affected the expression of the cell cycle-related microRNA (miR)-19a, along with its target gene PTEN, in G93A-SOD1 epSPCs. Overall our findings establish the significant impact of FM19G11-loaded NPs on the cellular pathways involved in self-renewal and proliferation in G93A-SOD1 epSPCs, thus providing an impetus to the design of novel tailored approaches to delay ALS disease progression.

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Zhang, Bin, Pang-hsien Tu, Farhad Abtahian, John Q. Trojanowski, and Virginia M. Y. Lee. "Neurofilaments and Orthograde Transport Are Reduced in Ventral Root Axons of Transgenic Mice that Express Human SOD1 with a G93A Mutation." Journal of Cell Biology 139, no. 5 (December 1, 1997): 1307–15. http://dx.doi.org/10.1083/jcb.139.5.1307.

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Mice engineered to express a transgene encoding a human Cu/Zn superoxide dismutase (SOD1) with a Gly93 → Ala (G93A) mutation found in patients who succumb to familial amyotrophic lateral sclerosis (FALS) develop a rapidly progressive and fatal motor neuron disease (MND) similar to amyotrophic lateral sclerosis (ALS). Hallmark ALS lesions such as fragmentation of the Golgi apparatus and neurofilament (NF)-rich inclusions in surviving spinal cord motor neurons as well as the selective degeneration of this population of neurons were also observed in these animals. Since the mechanism whereby mutations in SOD1 lead to MND remains enigmatic, we asked whether NF inclusions in motor neurons compromise axonal transport during the onset and progression of MND in a line of mice that contained ∼30% fewer copies of the transgene than the original G93A (Gurney et al., 1994). The onset of MND was delayed in these mice compared to the original G93A mice, but they developed the same neuropathologic abnormalities seen in the original G93A mice, albeit at a later time point with fewer vacuoles and more NF inclusions. Quantitative Western blot analyses showed a progressive decrease in the level of NF proteins in the L5 ventral roots of G93A mice and a concomitant reduction in axon caliber with the onset of motor weakness. By ∼200 d, both fast and slow axonal transports were impaired in the ventral roots of these mice coincidental with the appearance of NF inclusions and vacuoles in the axons and perikarya of vulnerable motor neurons. This is the first demonstration of impaired axonal transport in a mouse model of ALS, and we infer that similar impairments occur in authentic ALS. Based on the temporal correlation of these impairments with the onset of motor weakness and the appearance of NF inclusions and vacuoles in vulnerable motor neurons, the latter lesions may be the proximal cause of motor neuron dysfunction and degeneration in the G93A mice and in FALS patients with SOD1 mutations.

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7

Rey, Federica, Stefania Marcuzzo, Silvia Bonanno, Matteo Bordoni, Toniella Giallongo, Claudia Malacarne, Cristina Cereda, Gian Vincenzo Zuccotti, and Stephana Carelli. "LncRNAs Associated with Neuronal Development and Oncogenesis Are Deregulated in SOD1-G93A Murine Model of Amyotrophic Lateral Sclerosis." Biomedicines 9, no. 7 (July 13, 2021): 809. http://dx.doi.org/10.3390/biomedicines9070809.

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Amyotrophic Lateral Sclerosis (ALS) is a devastating neurodegenerative disease caused in 10% of cases by inherited mutations considered “familial”. An ever-increasing amount of evidence is showing a fundamental role for RNA metabolism in ALS pathogenesis, and long non-coding RNAs (lncRNAs) appear to play a role in ALS development. Here, we aim to investigate the expression of a panel of lncRNAs (linc-Enc1, linc–Brn1a, linc–Brn1b, linc-p21, Hottip, Tug1, Eldrr, and Fendrr) which could be implicated in early phases of ALS. Via Real-Time PCR, we assessed their expression in a murine familial model of ALS (SOD1-G93A mouse) in brain and spinal cord areas of SOD1-G93A mice in comparison with that of B6.SJL control mice, in asymptomatic (week 8) and late-stage disease (week 18). We highlighted a specific area and pathogenetic-stage deregulation in each lncRNA, with linc-p21 being deregulated in all analyzed tissues. Moreover, we analyzed the expression of their human homologues in SH-SY5Y-SOD1-WT and SH-SY5Y-SOD1-G93A, observing a profound alteration in their expression. Interestingly, the lncRNAs expression in our ALS models often resulted opposite to that observed for the lncRNAs in cancer. These evidences suggest that lncRNAs could be novel disease-modifying agents, biomarkers, or pathways affected by ALS neurodegeneration.

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8

Kuo, Jason J., Martijn Schonewille, Teepu Siddique, Annet N. A. Schults, Ronggen Fu, Peter R. Bär, Roberta Anelli, C. J. Heckman, and Alfons B. A. Kroese. "Hyperexcitability of Cultured Spinal Motoneurons From Presymptomatic ALS Mice." Journal of Neurophysiology 91, no. 1 (January 2004): 571–75. http://dx.doi.org/10.1152/jn.00665.2003.

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ALS (amyotrophic lateral sclerosis) is an adult-onset and deadly neurodegenerative disease characterized by a progressive and selective loss of motoneurons. Transgenic mice overexpressing a mutated human gene (G93A) coding for the enzyme SOD1 (Cu/Zn superoxide dismutase) develop a motoneuron disease resembling ALS in humans. In this generally accepted ALS model, we tested the electrophysiological properties of individual embryonic and neonatal spinal motoneurons in culture by measuring a wide range of electrical properties influencing motoneuron excitability during current clamp. There were no differences in the motoneuron resting potential, input conductance, action potential shape, or afterhyperpolarization between G93A and control motoneurons. The relationship between the motoneuron's firing frequency and injected current (f-I relation) was altered. The slope of the f-I relation and the maximal firing rate of the G93A motoneurons were much greater than in the control motoneurons. Differences in spontaneous synaptic input were excluded as a cause of increased excitability. This finding identifies a markedly elevated intrinsic electrical excitability in cultured embryonic and neonatal mutant G93A spinal motoneurons. We conclude that the observed intrinsic motoneuron hyperexcitability is induced by the SOD1 toxic gain-of-function through an aberration in the process of action potential generation. This hyperexcitability may play a crucial role in the pathogenesis of ALS as the motoneurons were cultured from presymptomatic mice.

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9

Zona, Cristina, Massimo Pieri, and Irene Carunchio. "Voltage-Dependent Sodium Channels in Spinal Cord Motor Neurons Display Rapid Recovery From Fast Inactivation in a Mouse Model of Amyotrophic Lateral Sclerosis." Journal of Neurophysiology 96, no. 6 (December 2006): 3314–22. http://dx.doi.org/10.1152/jn.00566.2006.

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Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease characterized by a substantial loss of motor neurons in the spinal cord, brain stem, and motor cortex. Previous evidence showed that in a mouse model of a familial form of ALS expressing high levels of the human mutated protein Cu,Zn superoxide dismutase (Gly93→Ala, G93A), the firing properties of single motor neurons are altered to induce neuronal hyperexcitability. To determine whether the functionality of the macroscopic voltage-dependent Na+ currents is modified in G93A motor neurons, in the present work their physiological properties were examined. The voltage-dependent sodium channels were studied in dissociated motor neurons in culture from nontransgenic mice (Control), from transgenic mice expressing high levels of the human wild-type protein [superoxide dismutase 1 (SOD1)], and from G93A mice, using the whole cell configuration of the patch-clamp recording technique. The voltage dependency of activation and of steady-state inactivation, the kinetics of fast inactivation and slow inactivation of the voltage-dependent Na+ channels were not modified in the mutated mice. Conversely, the recovery from fast inactivation was significantly faster in G93A motor neurons than that in Control and SOD1. The recovery from fast inactivation was still significantly faster in G93A motor neurons exposed for different times (3–48 h) and concentrations (5–500 μM) to edaravone, a free-radical scavenger. Clarification of the importance of these changes in membrane ion channel functionality may have diagnostic and therapeutic implications in the pathogenesis of ALS.

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10

Post, Julia, Vanessa Kogel, Anja Schaffrath, Philipp Lohmann, N. Jon Shah, Karl-Josef Langen, Dieter Willbold, Antje Willuweit, and Janine Kutzsche. "A Novel Anti-Inflammatory d-Peptide Inhibits Disease Phenotype Progression in an ALS Mouse Model." Molecules 26, no. 6 (March 13, 2021): 1590. http://dx.doi.org/10.3390/molecules26061590.

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Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease characterised by selective neuronal death in the brain stem and spinal cord. The cause is unknown, but an increasing amount of evidence has firmly certified that neuroinflammation plays a key role in ALS pathogenesis. Neuroinflammation is a pathological hallmark of several neurodegenerative disorders and has been implicated as driver of disease progression. Here, we describe a treatment study demonstrating the therapeutic potential of a tandem version of the well-known all-d-peptide RD2 (RD2RD2) in a transgenic mouse model of ALS (SOD1*G93A). Mice were treated intraperitoneally for four weeks with RD2RD2 vs. placebo. SOD1*G93A mice were tested longitudinally during treatment in various behavioural and motor coordination tests. Brain and spinal cord samples were investigated immunohistochemically for gliosis and neurodegeneration. RD2RD2 treatment in SOD1*G93A mice resulted not only in a reduction of activated astrocytes and microglia in both the brain stem and lumbar spinal cord, but also in a rescue of neurons in the motor cortex. RD2RD2 treatment was able to slow progression of the disease phenotype, especially the motor deficits, to an extent that during the four weeks treatment duration, no significant progression was observed in any of the motor experiments. Based on the presented results, we conclude that RD2RD2 is a potential therapeutic candidate against ALS.

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Linseman, Daniel A., Aimee N. Winter, and Heather M. Wilkins. "The 2-Oxoglutarate Carrier Is S-Nitrosylated in the Spinal Cord of G93A Mutant hSOD1 Mice Resulting in Disruption of Mitochondrial Glutathione Transport." Biomedicines 11, no. 1 (December 27, 2022): 61. http://dx.doi.org/10.3390/biomedicines11010061.

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Mitochondrial oxidative stress and dysfunction are strongly implicated in the pathogenesis of amyotrophic lateral sclerosis (ALS). Glutathione (GSH) is an endogenous antioxidant that exists as distinct cytosolic and mitochondrial pools. The status of the mitochondrial GSH pool is reliant on transport from the cytosol through the 2-oxoglutarate carrier (OGC), an inner membrane anion carrier. We have previously reported that the outer mitochondrial membrane protein, Bcl-2, directly binds GSH and is a key regulator of OGC-dependent mitochondrial GSH transport. Here, we show that G93A mutant SOD1 (Cu, Zn-superoxide dismutase) reduces the binding of GSH to Bcl-2 and disrupts mitochondrial GSH uptake in vitro. In the G93A mutant hSOD1 mouse model of ALS, mitochondrial GSH is significantly depleted in spinal cord of end-stage mice. Finally, we show that OGC is heavily S-nitrosylated in the spinal cord of end-stage mice and consequently, the GSH uptake capacity of spinal cord mitochondria isolated from these mutant mice is significantly diminished. Collectively, these findings suggest that spinal cord GSH depletion, particularly at the level of the mitochondria, plays a significant role in ALS pathogenesis induced by mutant SOD1. Furthermore, the depletion of mitochondrial GSH in the G93A mutant hSOD1 mouse model may be caused by the S-nitrosylation of OGC and the capacity of mutant SOD1 to disrupt the Bcl-2/GSH interaction, resulting in a disruption of mitochondrial GSH transport.

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12

Bonafede, Roberta, Ermanna Turano, Ilaria Scambi, Alice Busato, Pietro Bontempi, Federica Virla, Lorenzo Schiaffino, Pasquina Marzola, Bruno Bonetti, and Raffaella Mariotti. "ASC-Exosomes Ameliorate the Disease Progression in SOD1(G93A) Murine Model Underlining Their Potential Therapeutic Use in Human ALS." International Journal of Molecular Sciences 21, no. 10 (May 21, 2020): 3651. http://dx.doi.org/10.3390/ijms21103651.

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Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease characterized by progressive degeneration of motoneurons. To date, there is no effective treatment available. Exosomes are extracellular vesicles that play important roles in intercellular communication, recapitulating the effect of origin cells. In this study, we tested the potential neuroprotective effect of exosomes isolated from adipose-derived stem cells (ASC-exosomes) on the in vivo model most widely used to study ALS, the human SOD1 gene with a G93A mutation (SOD1(G93A)) mouse. Moreover, we compared the effect of two different routes of exosomes administration, intravenous and intranasal. The effect of exosomes administration on disease progression was monitored by motor tests and analysis of lumbar motoneurons and glial cells, neuromuscular junction, and muscle. Our results demonstrated that repeated administration of ASC-exosomes improved the motor performance; protected lumbar motoneurons, the neuromuscular junction, and muscle; and decreased the glial cells activation in treated SOD1(G93A) mice. Moreover, exosomes have the ability to home to lesioned ALS regions of the animal brain. These data contribute by providing additional knowledge for the promising use of ASC-exosomes as a therapy in human ALS.

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13

Gavin, Timothy P., Samuel L. Pittman, Elena S. Pak, and Alexander K. Murashov. "Gastrocnemius Muscle Capillarization Is Increased In SOD1-G93A Mice." Medicine & Science in Sports & Exercise 42 (May 2010): 121. http://dx.doi.org/10.1249/01.mss.0000386020.59007.39.

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14

Banerjee, Rebecca, R. Lee Mosley, Ashley D. Reynolds, Alok Dhar, Vernice Jackson-Lewis, Paul H. Gordon, Serge Przedborski, and Howard E. Gendelman. "Adaptive Immune Neuroprotection in G93A-SOD1 Amyotrophic Lateral Sclerosis Mice." PLoS ONE 3, no. 7 (July 23, 2008): e2740. http://dx.doi.org/10.1371/journal.pone.0002740.

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15

Wooley, Christine M., Roger B. Sher, Ajit Kale, Wayne N. Frankel, Gregory A. Cox, and Kevin L. Seburn. "Gait analysis detects early changes in transgenic SOD1(G93A) mice." Muscle & Nerve 32, no. 1 (2005): 43–50. http://dx.doi.org/10.1002/mus.20228.

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16

Renzini, Alessandra, Eva Pigna, Marco Rocchi, Alessia Cedola, Giuseppe Gigli, Viviana Moresi, and Dario Coletti. "Sex and HDAC4 Differently Affect the Pathophysiology of Amyotrophic Lateral Sclerosis in SOD1-G93A Mice." International Journal of Molecular Sciences 24, no. 1 (December 21, 2022): 98. http://dx.doi.org/10.3390/ijms24010098.

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Amyotrophic Lateral Sclerosis (ALS) is a devastating adult-onset neurodegenerative disease, with ineffective therapeutic options. ALS incidence and prevalence depend on the sex of the patient. Histone deacetylase 4 (HDAC4) expression in skeletal muscle directly correlates with the progression of ALS, pointing to the use of HDAC4 inhibitors for its treatment. Contrarily, we have found that deletion of HDAC4 in skeletal muscle worsened the pathological features of ALS, accelerating and exacerbating skeletal muscle loss and negatively affecting muscle innervations in male SOD1-G93A (SOD1) mice. In the present work, we compared SOD1 mice of both sexes with the aim to characterize ALS onset and progression as a function of sex differences. We found a global sex-dependent effects on disease onset and mouse lifespan. We further investigated the role of HDAC4 in SOD1 females with a genetic approach, and discovered morpho-functional effects on skeletal muscle, even in the early phase of the diseases. The deletion of HDAC4 decreased muscle function and exacerbated muscle atrophy in SOD1 females, and had an even more dramatic effect in males. Therefore, the two sexes must be considered separately when studying ALS.

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Khademullah, C. Sahara, Afif J. Aqrabawi, Kara M. Place, Zahra Dargaei, Xinyi Liang, Jessica C. Pressey, Simon Bedard, et al. "Cortical interneuron-mediated inhibition delays the onset of amyotrophic lateral sclerosis." Brain 143, no. 3 (March 1, 2020): 800–810. http://dx.doi.org/10.1093/brain/awaa034.

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Abstract Amyotrophic lateral sclerosis is a fatal disease resulting from motor neuron degeneration in the cortex and spinal cord. Cortical hyperexcitability is a hallmark feature of amyotrophic lateral sclerosis and is accompanied by decreased intracortical inhibition. Using electrophysiological patch-clamp recordings, we revealed parvalbumin interneurons to be hypoactive in the late pre-symptomatic SOD1*G93A mouse model of amyotrophic lateral sclerosis. We discovered that using adeno-associated virus-mediated delivery of chemogenetic technology targeted to increase the activity of the interneurons within layer 5 of the primary motor cortex, we were able to rescue intracortical inhibition and reduce pyramidal neuron hyperexcitability. Increasing the activity of interneurons in the layer 5 of the primary motor cortex was effective in delaying the onset of amyotrophic lateral sclerosis-associated motor deficits, slowing symptom progression, preserving neuronal populations, and increasing the lifespan of SOD1*G93A mice. Taken together, this study provides novel insights into the pathogenesis and treatment of amyotrophic lateral sclerosis.

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IGNACIO, S. "Effect of Neuroprotective Drugs on Gene Expression in G93A/SOD1 Mice." Annals of the New York Academy of Sciences 1053, no. 1 (August 1, 2005): 121–36. http://dx.doi.org/10.1196/annals.1344.010.

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von Lewinski, Friederike, Julia Fuchs, Bodo K. Vanselow, and Bernhard U. Keller. "Low Ca2+ buffering in hypoglossal motoneurons of mutant SOD1 (G93A) mice." Neuroscience Letters 445, no. 3 (November 2008): 224–28. http://dx.doi.org/10.1016/j.neulet.2008.08.084.

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Morimoto, Nobutoshi, Makiko Nagai, Yasuyuki Ohta, Kazunori Miyazaki, Tomoko Kurata, Mizuki Morimoto, Tetsuro Murakami, et al. "Increased autophagy in transgenic mice with a G93A mutant SOD1 gene." Brain Research 1167 (September 2007): 112–17. http://dx.doi.org/10.1016/j.brainres.2007.06.045.

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IGNACIO, SHEILA, DAN H. MOORE, ANDREW P. SMITH, and NANCY M. LEE. "Effect of Neuroprotective Drugs on Gene Expression in G93A/SOD1 Mice." Annals of the New York Academy of Sciences 1053, no. 1 (June 28, 2008): 121–36. http://dx.doi.org/10.1111/j.1749-6632.2005.tb00017.x.

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22

Hogg, Marion C., Mollie R. Mitchem, Hans-Georg König, and Jochen H. M. Prehn. "Caspase 6 has a protective role in SOD1 G93A transgenic mice." Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease 1862, no. 6 (June 2016): 1063–73. http://dx.doi.org/10.1016/j.bbadis.2016.03.006.

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23

Giagnorio, Eleonora, Claudia Malacarne, Paola Cavalcante, Letizia Scandiffio, Marco Cattaneo, Viviana Pensato, Cinzia Gellera, et al. "MiR-146a in ALS: Contribution to Early Peripheral Nerve Degeneration and Relevance as Disease Biomarker." International Journal of Molecular Sciences 24, no. 5 (February 27, 2023): 4610. http://dx.doi.org/10.3390/ijms24054610.

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Amyotrophic lateral sclerosis (ALS) is characterized by the progressive, irreversible loss of upper and lower motor neurons (UMNs, LMNs). MN axonal dysfunctions are emerging as relevant pathogenic events since the early ALS stages. However, the exact molecular mechanisms leading to MN axon degeneration in ALS still need to be clarified. MicroRNA (miRNA) dysregulation plays a critical role in the pathogenesis of neuromuscular diseases. These molecules represent promising biomarkers for these conditions since their expression in body fluids consistently reflects distinct pathophysiological states. Mir-146a has been reported to modulate the expression of the NFL gene, encoding the light chain of the neurofilament (NFL) protein, a recognized biomarker for ALS. Here, we analyzed miR-146a and Nfl expression in the sciatic nerve of G93A-SOD1 ALS mice during disease progression. The miRNA was also analyzed in the serum of affected mice and human patients, the last stratified relying on the predominant UMN or LMN clinical signs. We revealed a significant miR-146a increase and Nfl expression decrease in G93A-SOD1 peripheral nerve. In the serum of both ALS mice and human patients, the miRNA levels were reduced, discriminating UMN-predominant patients from the LMN ones. Our findings suggest a miR-146a contribution to peripheral axon impairment and its potential role as a diagnostic and prognostic biomarker for ALS.

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Stavrovskaya, А. V., D. N. Voronkov, E. A. Artyomova, B. V. Belugin, М. М. Shmarov, N. G. Yamshchikova, А. S. Gushchina, А. S. Olshansky, B. S. Naroditskiy, and S. N. Illarioshkin. "Genetic model of motor neuron disease in B6SJL-Tg mice: new data on the dynamics of motor symptoms and immunohistochemical manifestations of the neurodegenerative process." Neuromuscular Diseases 10, no. 3 (December 6, 2020): 63–73. http://dx.doi.org/10.17650/2222-8721-2020-10-3-63-73.

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Introduction. Over the past several decades, the study of mutations associated with motor neuron disease has led to the development of a number of transgenic animal models of motor neuron disease. One of the causes of the familial form of this disorder is mutations in the gene encoding Cu/Zn superoxide dismutase 1. The B6SJL-Tg (SOD1*G93A) mouse strain expresses a mutant form of human superoxide dismutase 1. Aim of study. To assess motor functions, dynamics of survival, and morphological changes in the spinal cord of transgenic B6SJL-Tg (SOD1*G93A) mice. Material and methods. In total, 31 animals have been studied. Starting from the age of 22 weeks, once every two weeks, the “open field” and “beam walking” motor tests were performed. The morphological changes in the spinal cord were evaluated at intermediate (26–35 weeks) and late stages (40–45 weeks). Neuronal proteins NeuN and PGP9.5, gliofibrillar protein, cyclonucleotide phosphatase (a marker of oligodendroglia) and a marker protein of microglia IBA1 were detected by immunohistochemistry; antibodies MTC02 to the outer membrane protein were used to detect mitochondria. Results. Motor problems appeared at the age of 24–26 weeks and steadily progressed; one could see consistent paresis of the hindlimbs, then the forelimbs, which was accompanied by general hypotrophy of the animals. There was a greater variability in the timing of symptom onset and life expectancy in males compared to females. The neurodegenerative process with damage to motor neurons was accompanied by the activation of micro- and astroglia. A sharp decrease in immunoreactivity to the mitochondrial marker MTC02 was found. Conclusion. The obtained results demonstrate new details of the development of a complex of motor and pathomorphological changes characteristic of motor neuron disease in B6SJL-Tg (SOD1*G93A) mice. Clarification of the fine dynamics of the neurodegenerative process in these animals is of great importance for monitoring the course of the disease during preclinical trials of new drugs and methods of gene therapy.

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Weydt, Patrick, Soyon Hong, Anke Witting, Thomas Möller, Nephi Stella, and Michel Kliot. "Cannabinol delays symptom onset in SOD1 (G93A) transgenic mice without affecting survival." Amyotrophic Lateral Sclerosis 6, no. 3 (January 2005): 182–84. http://dx.doi.org/10.1080/14660820510030149.

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Son, Marjatta, Scot C. Leary, Nadine Romain, Fabien Pierrel, Dennis R. Winge, Ronald G. Haller, and Jeffrey L. Elliott. "Isolated Cytochrome c Oxidase Deficiency in G93A SOD1 Mice Overexpressing CCS Protein." Journal of Biological Chemistry 283, no. 18 (March 11, 2008): 12267–75. http://dx.doi.org/10.1074/jbc.m708523200.

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Zhao, C. P., C. Zhang, S. N. Zhou, Y. M. Xie, Y. H. Wang, H. Huang, Y. C. Shang, et al. "Human mesenchymal stromal cells ameliorate the phenotype of SOD1-G93A ALS mice." Cytotherapy 9, no. 5 (2007): 414–26. http://dx.doi.org/10.1080/14653240701376413.

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Ma, Xiaoxing, Patrick C. Turnbull, Eli Prentice Crapper, Henan Wang, Anna Drannik, Fan Jiang, Sean Xia, and John Turnbull. "Cytosolic localization of Fox proteins in motor neurons of G93A SOD1 mice." Histochemistry and Cell Biology 145, no. 5 (January 2, 2016): 545–59. http://dx.doi.org/10.1007/s00418-015-1393-4.

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San Gil, Rebecca, Benjamin E. Clarke, Heath Ecroyd, Bernadett Kalmar, and Linda Greensmith. "Regional Differences in Heat Shock Protein 25 Expression in Brain and Spinal Cord Astrocytes of Wild-Type and SOD1G93A Mice." Cells 10, no. 5 (May 19, 2021): 1257. http://dx.doi.org/10.3390/cells10051257.

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Heterogeneity of glia in different CNS regions may contribute to the selective vulnerability of neuronal populations in neurodegenerative conditions such as amyotrophic lateral sclerosis (ALS). Here, we explored regional variations in the expression of heat shock protein 25 in glia under conditions of acute and chronic stress. Hsp27 (Hsp27; murine orthologue: Hsp25) fulfils a number of cytoprotective functions and may therefore be a possible therapeutic target in ALS. We identified a subpopulation of astrocytes in primary murine mixed glial cultures that expressed Hsp25. Under basal conditions, the proportion of Hsp25-positive astrocytes was twice as high in spinal cord cultures than in cortical cultures. To explore the physiological role of the elevated Hsp25 expression in spinal cord astrocytes, we exposed cortical and spinal cord glia to acute stress, using heat stress and pro-inflammatory stimuli. Surprisingly, we observed no stress-induced increase in Hsp25 expression in either cortical or spinal cord astrocytes. Similarly, exposure to endogenous stress, as modelled in glial cultures from SOD1 G93A-ALS mice, did not increase Hsp25 expression above that observed in astrocytes from wild-type mice. In vivo, Hsp25 expression was greater under conditions of chronic stress present in the spinal cord of SOD1 G93A mice than in wild-type mice, although this increase in expression is likely to be due to the extensive gliosis that occurs in this model. Together, these results show that there are differences in the expression of Hsp25 in astrocytes in different regions of the central nervous system, but Hsp25 expression is not upregulated under acute or chronic stress conditions.

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Marc, Gotkine, Rozenstein Leah, Einstein Ofira, Abramsky Oded, Argov Zohar, and Rosenmann Hanna. "Presymptomatic Treatment with Acetylcholinesterase Antisense Oligonucleotides Prolongs Survival in ALS (G93A-SOD1) Mice." BioMed Research International 2013 (2013): 1–5. http://dx.doi.org/10.1155/2013/845345.

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Objective. Previous research suggests that acetylcholinesterase (AChE) may be involved in ALS pathogenesis. AChE enzyme inhibitors can upregulate AChE transcription which in certain contexts can have deleterious (noncatalytic) effects, making them theoretically harmful in ALS, whilst AChE antisense-oligonucleotides (mEN101), which downregulate AChE may be beneficial. Our aim was to investigate whether downregulation of AChE using mEN101 is beneficial in an ALS mouse model.Methods. ALS (G93A-SOD1) mice received saline, mEN101, inverse-EN101, or neostigmine. Treatments were administered from 5 weeks. Disease-onset and survival were recorded. Additional mice were sacrificed for pathological analysis at 15 weeks of age. In a follow-up experiment treatment was started at the symptomatic stage at a higher dose.Results. mEN101 given at the presymptomatic (but not symptomatic) stage prolonged survival and attenuated motor-neuron loss in ALS mice. In contrast, neostigmine exacerbated the clinical parameters.Conclusions. These results suggest that AChE may be involved in ALS pathogenesis. The accelerated disease course with neostigmine suggests that any beneficial effects of mEN101 occur through a non-catalytic rather than cholinergic mechanism.

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Apolloni, Savina, Paola Fabbrizio, Susanna Amadio, Giulia Napoli, Mattia Freschi, Francesca Sironi, Paolo Pevarello, et al. "Novel P2X7 Antagonist Ameliorates the Early Phase of ALS Disease and Decreases Inflammation and Autophagy in SOD1-G93A Mouse Model." International Journal of Molecular Sciences 22, no. 19 (September 30, 2021): 10649. http://dx.doi.org/10.3390/ijms221910649.

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Amyotrophic lateral sclerosis (ALS) is a disease with a resilient neuroinflammatory component caused by activated microglia and infiltrated immune cells. How to successfully balance neuroprotective versus neurotoxic actions through the use of anti-inflammatory agents is still under debate. There has been a boost of awareness regarding the role of extracellular ATP and purinergic receptors in modulating the physiological and pathological mechanisms in the nervous system. Particularly in ALS, it is known that the purinergic ionotropic P2X7 receptor plays a dual role in disease progression by acting at different cellular and molecular levels. In this context, we previously demonstrated that the P2X7 receptor antagonist, brilliant blue G, reduces neuroinflammation and ameliorates some of the pathological features of ALS in the SOD1-G93A mouse model. Here, we test the novel, noncommercially available, and centrally permeant Axxam proprietary P2X7 antagonist, AXX71, in SOD1-G93A mice, by assessing some behavioral and molecular parameters, among which are disease progression, survival, gliosis, and motor neuron wealth. We demonstrate that AXX71 affects the early symptomatic phase of the disease by reducing microglia-related proinflammatory markers and autophagy without affecting the anti-inflammatory markers or motor neuron survival. Our results suggest that P2X7 modulation can be further investigated as a therapeutic strategy in preclinical studies, and exploited in ALS clinical trials.

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Chiu, Isaac M., Adam Chen, Yi Zheng, Bela Kosaras, Stefanos A. Tsiftsoglou, Timothy K. Vartanian, Robert H. Brown, and Michael C. Carroll. "T lymphocytes potentiate endogenous neuroprotective inflammation in a mouse model of ALS." Proceedings of the National Academy of Sciences 105, no. 46 (November 7, 2008): 17913–18. http://dx.doi.org/10.1073/pnas.0804610105.

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Amyotrophic Lateral Sclerosis (ALS) is an adult-onset, progressive, motor neuron degenerative disease, in which the role of inflammation is not well established. Innate and adaptive immunity were investigated in the CNS of the Superoxide Dismutase 1 (SOD1)G93A transgenic mouse model of ALS. CD4+ and CD8+ T cells infiltrated SOD1G93A spinal cords during disease progression. Cell-specific flow cytometry and gene expression profiling showed significant phenotypic changes in microglia, including dendritic cell receptor acquisition, and expression of genes linked to neuroprotection, cholesterol metabolism and tissue remodeling. Microglia dramatically up-regulated IGF-1 and down-regulated IL-6 expression. When mutant SOD1 mice were bred onto a TCRβ deficient background, disease progression was significantly accelerated at the symptomatic stage. In addition, microglia reactivity and IGF-1 levels were reduced in spinal cords of SOD1G93A (TCRβ−/−) mice. These results indicate that T cells play an endogenous neuroprotective role in ALS by modulating a beneficial inflammatory response to neuronal injury.

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Tarnopolsky, M. A., J. M. Bourgeois, R. Snow, S. Keys, B. D. Roy, J. M. Kwiecien, and J. Turnbull. "Histological assessment of intermediate- and long-term creatine monohydrate supplementation in mice and rats." American Journal of Physiology-Regulatory, Integrative and Comparative Physiology 285, no. 4 (October 2003): R762—R769. http://dx.doi.org/10.1152/ajpregu.00270.2003.

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Creatine monohydrate (CrM) supplementation appears to be relatively safe based on data from short-term and intermediate-term human studies and results from several therapeutic trials. The purpose of the current study was to characterize pathological changes after intermediate-term and long-term CrM supplementation in mice [healthy control and SOD1 (G93A) transgenic] and rats (prednisolone and nonprednisolone treated). Histological assessment (18-20 organs/tissues) was performed on G93A mice after 159 days, and in Sprague-Dawley rats after 365 days, of CrM supplementation (2% wt/wt) compared with control feed. Liver histology was also evaluated in CD-1 mice after 300 days of low-dose CrM supplementation (0.025 and 0.05 g · kg-1 · day-1) and in Sprague-Dawley rats after 52 days of CrM supplementation (2% wt/wt) with and without prednisolone. Areas of hepatitis were observed in the livers of the CrM-supplemented G93A mice ( P < 0.05), with no significant inflammatory lesions in any of the other 18-20 tissues/organs that were evaluated. The CD-1 mice also showed significant hepatic inflammatory lesions ( P < 0.05), yet there was no negative effect of CrM on liver histology in the Sprague-Dawley rats after intermediate-term or long-term supplementation nor was inflammation seen in any other tissues/organs ( P = not significant). Dietary CrM supplementation can induce inflammatory changes in the liver of mice, but not rats. The observed inflammatory changes in the murine liver must be considered in the evaluation of hepatic metabolism in CrM-supplemented mice. Species differences must be considered in the evaluation of toxicological and physiological studies.

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Jiang, F., W. P. Li, J. Kwiecien, and J. Turnbull. "A Study of the Purine Derivative AIT-082 in G93A SOD1 Transgenic Mice." International Journal of Immunopathology and Pharmacology 19, no. 3 (July 2006): 489–98. http://dx.doi.org/10.1177/039463200601900304.

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Sasaki, Shoichi, Hitoshi Warita, Koji Abe, Takashi Komori, and Makoto Iwata. "EAAT1 and EAAT2 immunoreactivity in transgenic mice with a G93A mutant SOD1 gene." Neuroreport 12, no. 7 (May 2001): 1359–62. http://dx.doi.org/10.1097/00001756-200105250-00014.

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Bonfanti, Elisabetta, Tiziana Bonifacino, Stefano Raffaele, Marco Milanese, Erica Morgante, Giambattista Bonanno, Maria P. Abbracchio, and Marta Fumagalli. "Abnormal Upregulation of GPR17 Receptor Contributes to Oligodendrocyte Dysfunction in SOD1 G93A Mice." International Journal of Molecular Sciences 21, no. 7 (March 31, 2020): 2395. http://dx.doi.org/10.3390/ijms21072395.

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Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease characterized by progressive loss of motor neurons (MN). Importantly, MN degeneration is intimately linked to oligodendrocyte dysfunction and impaired capacity of oligodendrocyte precursor cells (OPCs) to regenerate the myelin sheath enwrapping and protecting neuronal axons. Thus, improving OPC reparative abilities represents an innovative approach to counteract MN loss. A pivotal regulator of OPC maturation is the P2Y-like G protein-coupled receptor 17 (GPR17), whose role in ALS has never been investigated. In other models of neurodegeneration, an abnormal increase of GPR17 has been invariably associated to myelin defects and its pharmacological manipulation succeeded in restoring endogenous remyelination. Here, we analyzed GPR17 alterations in the SOD1G93A ALS mouse model and assessed in vitro whether this receptor could be targeted to correct oligodendrocyte alterations. Western-blot and immunohistochemical analyses showed that GPR17 protein levels are significantly increased in spinal cord of ALS mice at pre-symptomatic stage; this alteration is exacerbated at late symptomatic phases. Concomitantly, mature oligodendrocytes degenerate and are not successfully replaced. Moreover, OPCs isolated from spinal cord of SOD1G93A mice display defective differentiation compared to control cells, which is rescued by treatment with the GPR17 antagonist montelukast. These data open novel therapeutic perspectives for ALS management.

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Wier, Christopher G., Alexander E. Crum, Anthony B. Reynolds, Chitra C. Iyer, Deepti Chugh, Marilly S. Palettas, Patrick L. Heilman, David M. Kline, W. David Arnold, and Stephen J. Kolb. "Muscle contractility dysfunction precedes loss of motor unit connectivity in SOD1(G93A) mice." Muscle & Nerve 59, no. 2 (December 21, 2018): 254–62. http://dx.doi.org/10.1002/mus.26365.

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Han, Soyoung, Jong-Ryoul Choi, Ki Soon Shin, and Shin Jung Kang. "Resveratrol upregulated heat shock proteins and extended the survival of G93A-SOD1 mice." Brain Research 1483 (November 2012): 112–17. http://dx.doi.org/10.1016/j.brainres.2012.09.022.

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Seo, Ji-Seon, In-Sun Baek, Yea-Hyun Leem, Tae-Kyung Kim, Yearin Cho, Soo Min Lee, Yang Hae Park, and Pyung-Lim Han. "SK-PC-B70M alleviates neurologic symptoms in G93A-SOD1 amyotrophic lateral sclerosis mice." Brain Research 1368 (January 2011): 299–307. http://dx.doi.org/10.1016/j.brainres.2010.10.048.

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40

Fogarty, Matthew J., Erica W. H. Mu, Nickolas A. Lavidis, Peter G. Noakes, and Mark C. Bellingham. "Size‐Dependent Vulnerability of Lumbar Motor Neuron Dendritic Degeneration in SOD1 G93A Mice." Anatomical Record 303, no. 5 (September 30, 2019): 1455–71. http://dx.doi.org/10.1002/ar.24255.

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41

Peggion, Caterina, Valeria Scalcon, Maria Lina Massimino, Kelly Nies, Raffaele Lopreiato, Maria Pia Rigobello, and Alessandro Bertoli. "SOD1 in ALS: Taking Stock in Pathogenic Mechanisms and the Role of Glial and Muscle Cells." Antioxidants 11, no. 4 (March 23, 2022): 614. http://dx.doi.org/10.3390/antiox11040614.

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Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disorder characterized by the loss of motor neurons in the brain and spinal cord. While the exact causes of ALS are still unclear, the discovery that familial cases of ALS are related to mutations in the Cu/Zn superoxide dismutase (SOD1), a key antioxidant enzyme protecting cells from the deleterious effects of superoxide radicals, suggested that alterations in SOD1 functionality and/or aberrant SOD1 aggregation strongly contribute to ALS pathogenesis. A new scenario was opened in which, thanks to the generation of SOD1 related models, different mechanisms crucial for ALS progression were identified. These include excitotoxicity, oxidative stress, mitochondrial dysfunctions, and non-cell autonomous toxicity, also implicating altered Ca2+ metabolism. While most of the literature considers motor neurons as primary target of SOD1-mediated effects, here we mainly discuss the effects of SOD1 mutations in non-neuronal cells, such as glial and skeletal muscle cells, in ALS. Attention is given to the altered redox balance and Ca2+ homeostasis, two processes that are strictly related with each other. We also provide original data obtained in primary myocytes derived from hSOD1(G93A) transgenic mice, showing perturbed expression of Ca2+ transporters that may be responsible for altered mitochondrial Ca2+ fluxes. ALS-related SOD1 mutants are also responsible for early alterations of fundamental biological processes in skeletal myocytes that may impinge on skeletal muscle functions and the cross-talk between muscle cells and motor neurons during disease progression.

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Debye, Berthold, Lena Schmülling, Lepu Zhou, Gabriele Rune, Cordian Beyer, and Sonja Johann. "Neurodegeneration and NLRP3 inflammasome expression in the anterior thalamus of SOD1(G93A) ALS mice." Brain Pathology 28, no. 1 (March 22, 2017): 14–27. http://dx.doi.org/10.1111/bpa.12467.

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Sawada, Atsushi, Shanshan Wang, Minyu Jian, Joseph Leem, Jesse Wackerbarth, Junji Egawa, Jan M. Schilling, et al. "Neuron‐targeted caveolin‐1 improves neuromuscular function and extends survival in SOD1 G93A mice." FASEB Journal 33, no. 6 (March 20, 2019): 7545–54. http://dx.doi.org/10.1096/fj.201802652rr.

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Trumbull, Kari A., Donna McAllister, Mandi M. Gandelman, Whitney Y. Fung, Thomas Lew, Lucy Brennan, Nathan Lopez, Jeff Morré, Balaraman Kalyanaraman, and Joseph S. Beckman. "Diapocynin and apocynin administration fails to significantly extend survival in G93A SOD1 ALS mice." Neurobiology of Disease 45, no. 1 (January 2012): 137–44. http://dx.doi.org/10.1016/j.nbd.2011.07.015.

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Deitch, Jeffrey S., Guillermo M. Alexander, Luis Del Valle, and Terry D. Heiman-Patterson. "GLT-1 glutamate transporter levels are unchanged in mice expressing G93A human mutant SOD1." Journal of the Neurological Sciences 193, no. 2 (January 2002): 117–26. http://dx.doi.org/10.1016/s0022-510x(01)00656-6.

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Ahn, Suk-Won, Gye Sun Jeon, Myung-Jin Kim, Jee-Heun Shon, Jee-Eun Kim, Je-Young Shin, Sung-Min Kim, et al. "Neuroprotective effects of JGK-263 in transgenic SOD1-G93A mice of amyotrophic lateral sclerosis." Journal of the Neurological Sciences 340, no. 1-2 (May 2014): 112–16. http://dx.doi.org/10.1016/j.jns.2014.03.008.

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47

Lee, Junghee, Hoon Ryu, and Neil W. Kowall. "Motor neuronal protection by l-arginine prolongs survival of mutant SOD1 (G93A) ALS mice." Biochemical and Biophysical Research Communications 384, no. 4 (July 2009): 524–29. http://dx.doi.org/10.1016/j.bbrc.2009.05.015.

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Lev, Nirit, Debby Ickowicz, Yael Barhum, Eldad Melamed, and Daniel Offen. "DJ-1 Changes in G93A-SOD1 Transgenic Mice: Implications for Oxidative Stress in ALS." Journal of Molecular Neuroscience 38, no. 2 (August 19, 2008): 94–102. http://dx.doi.org/10.1007/s12031-008-9138-7.

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Nagy, Maria, Wayne A. Fenton, Di Li, Krystyna Furtak, and Arthur L. Horwich. "Extended survival of misfolded G85R SOD1-linked ALS mice by transgenic expression of chaperone Hsp110." Proceedings of the National Academy of Sciences 113, no. 19 (April 25, 2016): 5424–28. http://dx.doi.org/10.1073/pnas.1604885113.

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Recent studies have indicated that mammalian cells contain a cytosolic protein disaggregation machinery comprised of Hsc70, DnaJ homologs, and Hsp110 proteins, the last of which acts to accelerate a rate-limiting step of nucleotide exchange of Hsc70. We tested the ability of transgenic overexpression of a Thy1 promoter-driven human Hsp110 protein, HspA4L (Apg1), in neuronal cells of a transgenic G85R SOD1YFP ALS mouse strain to improve survival. Notably, G85R is a mutant version of Cu/Zn superoxide dismutase 1 (SOD1) that is unable to reach native form and that is prone to aggregation, with prominent YFP-fluorescent aggregates observed in the motor neurons of the transgenic mice as early as 1 mo of age. The several-fold overexpression of Hsp110 in motor neurons of these mice was associated with an increased median survival from ∼5.5 to 7.5 mo and increased maximum survival from 6.5 to 12 mo. Improvement of survival was also observed for a G93A mutant SOD1 ALS strain. We conclude that neurodegeneration associated with cytosolic misfolding and aggregation can be ameliorated by overexpression of Hsp110, likely enhancing the function of a cytosolic disaggregation machinery.

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Scaricamazza, Silvia, Illari Salvatori, Susanna Amadio, Valentina Nesci, Alessio Torcinaro, Giacomo Giacovazzo, Aniello Primiano, et al. "Repurposing of Trimetazidine for amyotrophic lateral sclerosis: A study in SOD1 ^G93A mice." British Journal of Pharmacology 179, no. 8 (January 13, 2022): 1732–52. http://dx.doi.org/10.1111/bph.15738.

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