Academic literature on the topic 'NPC1a'
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Journal articles on the topic "NPC1a"
Phillips, S. E., E. A. Woodruff, P. Liang, M. Patten, and K. Broadie. "Neuronal Loss of Drosophila NPC1a Causes Cholesterol Aggregation and Age-Progressive Neurodegeneration." Journal of Neuroscience 28, no. 26 (June 25, 2008): 6569–82. http://dx.doi.org/10.1523/jneurosci.5529-07.2008.
Full textBialistoky, Tzofia, Diane Manry, Peyton Smith, Christopher Ng, Yunah Kim, Sol Zamir, Victoria Moyal, et al. "Functional analysis of Niemann-Pick disease type C family protein, NPC1a, in Drosophila melanogaster." Development 146, no. 10 (May 15, 2019): dev168427. http://dx.doi.org/10.1242/dev.168427.
Full textAntipova, Veronica, Lisa-Marie Steinhoff, Carsten Holzmann, Arndt Rolfs, Carlos Junior Hempel, Martin Witt, and Andreas Wree. "Organ Weights in NPC1 Mutant Mice Partly Normalized by Various Pharmacological Treatment Approaches." International Journal of Molecular Sciences 24, no. 1 (December 29, 2022): 573. http://dx.doi.org/10.3390/ijms24010573.
Full textKo, Dennis C., Michael D. Gordon, Janet Y. Jin, and Matthew P. Scott. "Dynamic Movements of Organelles Containing Niemann-Pick C1 Protein: NPC1 Involvement in Late Endocytic Events." Molecular Biology of the Cell 12, no. 3 (March 2001): 601–14. http://dx.doi.org/10.1091/mbc.12.3.601.
Full textCawley, Niamh X., Anna T. Lyons, Daniel Abebe, Rachel Luke, Julia Yerger, Rebecca Telese, Christopher A. Wassif, Joan E. Bailey-Wilson, and Forbes D. Porter. "Complex N-Linked Glycosylation: A Potential Modifier of Niemann–Pick Disease, Type C1 Pathology." International Journal of Molecular Sciences 23, no. 9 (May 3, 2022): 5082. http://dx.doi.org/10.3390/ijms23095082.
Full textDardis, Andrea, Stefania Zampieri, Cinzia Gellera, Rosalba Carrozzo, Silvia Cattarossi, Paolo Peruzzo, Rosalia Dariol, et al. "Molecular Genetics of Niemann–Pick Type C Disease in Italy: An Update on 105 Patients and Description of 18 NPC1 Novel Variants." Journal of Clinical Medicine 9, no. 3 (March 3, 2020): 679. http://dx.doi.org/10.3390/jcm9030679.
Full textCastillo, Joseph J., David Jelinek, Hao Wei, Nicholas P. Gannon, Roger A. Vaughan, L. John Horwood, F. John Meaney, et al. "The Niemann-Pick C1 gene interacts with a high-fat diet to promote weight gain through differential regulation of central energy metabolism pathways." American Journal of Physiology-Endocrinology and Metabolism 313, no. 2 (August 1, 2017): E183—E194. http://dx.doi.org/10.1152/ajpendo.00369.2016.
Full textLi, Xiaochun, Piyali Saha, Jian Li, Günter Blobel, and Suzanne R. Pfeffer. "Clues to the mechanism of cholesterol transfer from the structure of NPC1 middle lumenal domain bound to NPC2." Proceedings of the National Academy of Sciences 113, no. 36 (August 22, 2016): 10079–84. http://dx.doi.org/10.1073/pnas.1611956113.
Full textMeneses-Salas, Elsa, Ana García-Melero, Patricia Blanco-Muñoz, Jaimy Jose, Marie-Sophie Brenner, Albert Lu, Francesc Tebar, Thomas Grewal, Carles Rentero, and Carlos Enrich. "Selective Degradation Permits a Feedback Loop Controlling Annexin A6 and Cholesterol Levels in Endolysosomes of NPC1 Mutant Cells." Cells 9, no. 5 (May 7, 2020): 1152. http://dx.doi.org/10.3390/cells9051152.
Full textGläser, Anne, Franziska Hammerl, Markus H. Gräler, Sina M. Coldewey, Christin Völkner, Moritz J. Frech, Fan Yang, et al. "Identification of Brain-Specific Treatment Effects in NPC1 Disease by Focusing on Cellular and Molecular Changes of Sphingosine-1-Phosphate Metabolism." International Journal of Molecular Sciences 21, no. 12 (June 24, 2020): 4502. http://dx.doi.org/10.3390/ijms21124502.
Full textDissertations / Theses on the topic "NPC1a"
Clark, Emily. "Involvement of the lysosomal protein NPC1 in the pathogenesis of Huntington's disease." Thesis, Cardiff University, 2017. http://orca.cf.ac.uk/111724/.
Full textAwan, Sara. "Rôle de Wnt5a dans la fonction lysosomale, l’accumulation intracellulaire du cholestérol, et l’athérosclérose." Thesis, Strasbourg, 2019. http://www.theses.fr/2019STRAJ022.
Full textWe identified the Wnt ligand, Wnt5a, as a member of the nutrient/energy/stress sensor, mTORC1 scaffolding complex, which drives lysosomal function and promotes cholesterol trafficking. By decreasing mTORC1 activity and by activating the autophagy-lysosomal axis, Wnt5a senses changes in dietary cholesterol supply, promotes endosomal/lysosomal (LELs) cholesterol egress to the endoplasmic reticulum (ER), and protects against atherosclerosis. Moreover, Wnt5a binds cholesterol-rich membranes and specifically interacts with two lysosomal proteins Niemann–Pick C1 and Niemann–Pick C2 that regulate cholesterol export from LELs. Consequently, absence of Wnt5a decoupled mTORC1 from variations in LELs sterol levels, and this resulted in accumulation of large intracellular inclusion bodies, large LELs and low ER cholesterol
Landry, Chandra. "Phosphatidylcholine Metabolism and ACAT Affect the Trafficking of LDL-derived Free Cholesterol in Cholesterol-loaded CHO Cells." Thèse, Université d'Ottawa / University of Ottawa, 2012. http://hdl.handle.net/10393/23087.
Full textPalladino, G., S. Loizzo, A. Fortuna, S. Canterini, F. Palombi, R. P. Erickson, F. Mangia, and M. T. Fiorenza. "Visual evoked potentials of Niemann-Pick type C1 mice reveal an impairment of the visual pathway that is rescued by 2-hydroxypropyl-ẞ-cyclodextrin." BioMed Central, 2015. http://hdl.handle.net/10150/610327.
Full textAlmeida, Marcela Lopes de. "Niemann pick tipo C: caracterização fenotípica e genotípica de uma casuística brasileira." Universidade de São Paulo, 2016. http://www.teses.usp.br/teses/disponiveis/17/17160/tde-06062017-165658/.
Full textNiemann-Pick type C (NPC) is a lysosomal storage disease caused by abnormal unesterified cholesterol trafficking, resulting from biallelic changes in NPC1 or NPC2 genes, both defining an autosomal recessive progressive and irreversible disease characterized by visceral, neurological and psychiatric manifestations, not necessarily combined. In order to describe the phenotypic and genotypic characteristics of patients with NPC, this work aimed to report demographic data, clinical forms classified by age, neurological and psychiatric signs and symptoms, brain magnetic resonance imaging (MRI) and abdominal ultrasound findings, Filipin test, the gene mutations, and the treatment with N-butyldeoxynojirimycin (Miglustat). A series of 12 patients were studied, treated between 2000-2014, by review of medical records of the Neurogenetics Clinic at the Hospital of Clinics, Ribeirão Preto Medical School, Brazil. There were 7 women and 5 men, mean age 20 years (from 2 to 42); 10 caucasian and 2 mulattos, coming from three Brazilian states: São Paulo, Mato Grosso do Sul and Minas Gerais. Infantile, Juvenile and adult clinical forms were identified. The age of the first neurological or psichiatric symptoms occurred between 1 and 27 years (mean 9.5). Among the visceral findings, two patients were asymptomatic, and the others had prolonged jaundice / cholestasis, hepatomegaly and splenomegaly. All patients had at different times of evolution symptoms, such as paralysis of vertical gaze, ataxia/falls, epilepsy, myoclonus, dystonia, dysarthria, dysphagia, muscle weakness, spasticity, cognitive decline/dementia, psychotic symptoms, school delay, disorders behavior, gelastic cataplexy and neonatal hypotonia. Age at diagnosis ranged from 0 to 41 years, with a mean of 14.5 years. The interval between the first signs of the disease and the onset of treatment ranged from zero to 14 years, with an average of 5.3 years. Filipin test resulted six positive and six variant form. The MRI scans showed three different types of changes: brain atrophy in 6 cases, cerebellar atrophy in 7 and demyelination in 7. Abdominal ultrasound revealed 8 patients with hepatomegaly, 10 with splenomegaly and 8 hepatosplenomegaly. The results of the molecular genetic testing on 11 patients showed changes in NPC1 gene and a patient did not have the result. Mutation c.3104C>T was more frequent in eight patients; c.3548G>A, of uncertain significance in a patient, and other mutations found: c.3493G>A and c.3019C>G. Treatment with N-butyl deoxynojirimycin (Miglustat) was carried by all patients; the time between diagnosis and beginning of the treatment ranged from 0 to 9, with an average of 2.9 years. We conclude that NPC disease registry should be done through a collection of detailed and continuous data because their phenotypic and genotypic heterogeneity suggest an underestimated number of cases, not only for its rarity but also by unawareness about the disease, and the fact that there are few published studies. The early recognition, coupled with appropriate clinical management, may slow the progression of the disease and increase life expectancy of patients.
Bruchez, Anna. "Niemann-Pick C1 Is Essential for Ebola Virus Infection and a Target of Small Molecule Inhibitors." Thesis, Harvard University, 2012. http://dissertations.umi.com/gsas.harvard:10654.
Full textВецнер, Юлана Ігорівна. "Технологія NPCа-добрив з використанням карбаміду та фосфоритів з низьким вмістом фосфору (V) оксиду." Thesis, НТУ "ХПІ", 2015. http://repository.kpi.kharkov.ua/handle/KhPI-Press/18483.
Full textDissertation for degree of candidate of technical sciences, specialty 05.17.01 - Technology of inorganic substances. - National Technical University "Kharkov Polytechnic Institute", Kharkov, 2015. Dissertation is devoted to development of scientific bases of technology NPCa - involving phosphorus fertilizer raw materials with low content of phosphorus (V) oxide. For the first time on the basis of thermodynamic calculations and experimental studies, X-ray analysis of a list of reactions in the process of interaction with the urea nitrogen - acid extract (NAE). Defined technological parameters of the interaction of urea with nitrogen - acid extract and ammoniation process NPCa-fertilizers and developed concept that allows you to receive a number of complex NPCa-fertilizer with trace elements of non-waste technology using phosphorus-containing raw materials in Ukraine are low in phosphorus (V) oxide. It was found that the interaction of urea with the nitrogen - acid extract is happening in the kinetic region, established kinetic model of the process, found the rate constants, activation energy. A mathematical model that allows you to calculate the technological parameters of the process and the chemical synthesis reactor. The resulting complex NPCa - fertilizer with trace elements due to the high content of water-soluble and digestible forms of Р₂О₅ and CaO in the agro-chemical tests at the National Scientific Center "Institute for Soil Science and Agricultural Chemistry named after Sokolovsky" has shown an increase in crop yields of up to 12 ÷ 30 %. Prove their economic viability.
Dani, Vincent. "Étude du maintien et de la rupture de l'association symbiotique Cnidaire-Dinoflagellés : approches cellulaires et moléculaires chez l'anémone de mer Anemonia viridis." Thesis, Nice, 2015. http://www.theses.fr/2015NICE4105/document.
Full textThe trophic endosymbiosis interaction between a cnidarian host and its photosynthetic dinoflagellatessymbionts form the basis of coral reef ecosystems. Cnidarians host their symbionts in gastrodermis cells, in a phagocytosis-derived vacuole. Establishment and maintenance of the symbiotic interaction depend on an intimate molecular communication between the two partners. However, environmental and/or anthropogenic disturbances can lead to the breakdown of the symbiotic association, which is responsible for the worldwide decline of coral reefs. The main objectives of my research project are to improve the knowledge regarding symbiosis maintenance and disruption mechanisms, but also to define the molecular key players involved at the symbiotic interface in the sea anemone, Anemonia viridis. First, we have described the cellular mechanisms involved in the different types of symbiosis breackdown. Meanwhile, the characterization of npc1 and npc2 genes (involved in endosomal sterol transport), showed a duplication and a sub-functionalization of the npc2d gene. Both NPC1 and NPC2 proteins are expressed around symbionts. We therefore suggest that the duplicated protein NPC2-d is a biomarker of symbiosis health and that NPC1 protein is a marker of the perisymbiotic membrane. We then developed a protocol to characterize the proteome of the symbiotic interface between the two symbiotic partners. The newly-identified symbiotic key players will increase the general knowledge on the symbiotic interaction and its regulation during both stable and bleaching conditions
Вецнер, Юлана Ігорівна. "Технологія NPCа-добрив з використанням карбаміду та фосфоритів з низьким вмістом фосфору (V) оксиду." Thesis, НТУ "ХПІ", 2015. http://repository.kpi.kharkov.ua/handle/KhPI-Press/18482.
Full textDissertation for degree of candidate of technical sciences, specialty 05.17.01 - Technology of inorganic substances. - National Technical University "Kharkov Polytechnic Institute", Kharkov, 2015. Dissertation is devoted to development of scientific bases of technology NPCa - involving phosphorus fertilizer raw materials with low content of phosphorus (V) oxide. For the first time on the basis of thermodynamic calculations and experimental studies, X-ray analysis of a list of reactions in the process of interaction with the urea nitrogen - acid extract (NAE). Defined technological parameters of the interaction of urea with nitrogen - acid extract and ammoniation process NPCa-fertilizers and developed concept that allows you to receive a number of complex NPCa-fertilizer with trace elements of non-waste technology using phosphorus-containing raw materials in Ukraine are low in phosphorus (V) oxide. It was found that the interaction of urea with the nitrogen - acid extract is happening in the kinetic region, established kinetic model of the process, found the rate constants, activation energy. A mathematical model that allows you to calculate the technological parameters of the process and the chemical synthesis reactor. The resulting complex NPCa - fertilizer with trace elements due to the high content of water-soluble and digestible forms of Р₂О₅ and CaO in the agro-chemical tests at the National Scientific Center "Institute for Soil Science and Agricultural Chemistry named after Sokolovsky" has shown an increase in crop yields of up to 12 ÷ 30 %. Prove their economic viability.
Lundmark, Per Erik. "Genetic and Genomic Analysis of DNA Sequence Variation." Doctoral thesis, Uppsala universitet, Molekylär medicin, 2011. http://urn.kb.se/resolve?urn=urn:nbn:se:uu:diva-158486.
Full textBooks on the topic "NPC1a"
Smith, Eric H. NPCA field guide to structural pests. [Dunn Loring, Va.]: NPCA, 1992.
Find full textInc, Industry Insights, and National Paint and Coatings Association., eds. 1993 NPCA fringe benefits survey. Washington, D.C. (1500 Rhode Island Ave., NW, Washington, DC 20005-5597): National Paint and Coatings Association, 1993.
Find full textSedel, Frédéric. Niemann-Pick Disease Type C. Oxford University Press, 2016. http://dx.doi.org/10.1093/med/9780199972135.003.0053.
Full textNational Park and Conservation Association. Investing in Park Futures - The National Park System Plan: A Blueprint for Tomorrow- NPCA - Executive Summary (Executive Summary). Natl Parks & Conservation Association, 1988.
Find full textBook chapters on the topic "NPC1a"
Gooch, Jan W. "NPCA." In Encyclopedic Dictionary of Polymers, 491. New York, NY: Springer New York, 2011. http://dx.doi.org/10.1007/978-1-4419-6247-8_8006.
Full textMiles, John C. "NPCA: Seventy-five and Working." In Guardians of the Parks, 321–33. Boca Raton: Taylor & Francis, 2022. http://dx.doi.org/10.4324/9781315831176-13.
Full textCologna, Stephanie M., and Avia Rosenhouse-Dantsker. "Insights into the Molecular Mechanisms of Cholesterol Binding to the NPC1 and NPC2 Proteins." In Advances in Experimental Medicine and Biology, 139–60. Cham: Springer International Publishing, 2019. http://dx.doi.org/10.1007/978-3-030-14265-0_8.
Full textZampieri, Stefania, Bruno Bembi, Natalia Rosso, Mirella Filocamo, and Andrea Dardis. "Treatment of Human Fibroblasts Carrying NPC1 Missense Mutations with MG132 Leads to an Improvement of Intracellular Cholesterol Trafficking." In JIMD Reports, 59–69. Berlin, Heidelberg: Springer Berlin Heidelberg, 2011. http://dx.doi.org/10.1007/8904_2011_49.
Full text"NPCA." In Encyclopedic Dictionary of Polymers, 660. New York, NY: Springer New York, 2007. http://dx.doi.org/10.1007/978-0-387-30160-0_7864.
Full textTurner-Stokes, Nyein, and Halliwell. "Northwick Park Care Needs Assessment (NPCNA)." In A Compendium of Tests, Scales and Questionnaires, 580–83. Psychology Press, 2020. http://dx.doi.org/10.4324/9781003076391-160.
Full textConference papers on the topic "NPC1a"
Jankowski, Stanisław, Zbigniew Szymański, Uladzimir Dziomin, Vladimir Golovko, and Aleksy Barcz. "Deep learning classifier based on NPCA and orthogonal feature selection." In Photonics Applications in Astronomy, Communications, Industry, and High-Energy Physics Experiments 2016, edited by Ryszard S. Romaniuk. SPIE, 2016. http://dx.doi.org/10.1117/12.2249848.
Full textYing, Gao, Dong Hongbin, Ou Shifeng, Xu Jindong, and Cai Zhuoran. "Optimal Variable Momentum Factor Algorithm for NPCA in Blind Source Separation." In 2021 IEEE 18th International Conference on Mobile Ad Hoc and Smart Systems (MASS). IEEE, 2021. http://dx.doi.org/10.1109/mass52906.2021.00027.
Full textXin Hao, Kwok-wai Ma, Jia Zhao, and Xin-Yu Sun. "Design of NPC1 power stack beyond megawatt for 1500V solar inverter application." In 2017 IEEE 3rd International Future Energy Electronics Conference and ECCE Asia (IFEEC 2017 - ECCE Asia). IEEE, 2017. http://dx.doi.org/10.1109/ifeec.2017.7992204.
Full textHao, Xin, Kwok-wai Ma, Yong Yang, and Jia Zhao. "1500V solar inverter at megawatts level in NPC1 topology enabled by high-density IGBT module." In TENCON 2016 - 2016 IEEE Region 10 Conference. IEEE, 2016. http://dx.doi.org/10.1109/tencon.2016.7848444.
Full textSlawinski, Maximilian, Benjamin Sahan, and Uwe Jansen. "Evaluation of a NPC1 phase leg built from three standard IGBT modules for 1500 VDC photovoltaic central inverters up to 800 kVA." In 2016 18th European Conference on Power Electronics and Applications (EPE'16 ECCE Europe). IEEE, 2016. http://dx.doi.org/10.1109/epe.2016.7695333.
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