Academic literature on the topic 'NPC1a'
Create a spot-on reference in APA, MLA, Chicago, Harvard, and other styles
Consult the lists of relevant articles, books, theses, conference reports, and other scholarly sources on the topic 'NPC1a.'
Next to every source in the list of references, there is an 'Add to bibliography' button. Press on it, and we will generate automatically the bibliographic reference to the chosen work in the citation style you need: APA, MLA, Harvard, Chicago, Vancouver, etc.
You can also download the full text of the academic publication as pdf and read online its abstract whenever available in the metadata.
Journal articles on the topic "NPC1a"
1
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 text
2
Bialistoky, 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 text
3
Antipova, 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 textAbstract:
Niemann-Pick Type C1 (NPC1, MIM 257220) is a rare, progressive, lethal, inherited autosomal-recessive endolysosomal storage disease caused by mutations in the NPC1 leading to intracellular lipid storage. We analyzed mostly not jet known alterations of the weights of 14 different organs in the BALB/cNctr-Npc1m1N/-J Jackson Npc1 mice in female and male Npc1+/+ and Npc1−/− mice under various treatment strategies. Mice were treated with (i) no therapy, (ii) vehicle injection, (iii) a combination of miglustat, allopregnanolone, and 2-hydroxypropyl-ß-cyclodextrin (HPßCD), (iv) miglustat, and (v) HPßCD alone starting at P7 and repeated weekly throughout life. The 12 respective male and female wild-type mice groups were evaluated in parallel. In total, 351 mice (176 Npc1+/+, 175 Npc1−/−) were dissected at P65. In both sexes, the body weights of None and Sham Npc1−/− mice were lower than those of respective Npc1+/+ mice. The influence of the Npc1 mutation and/or sex on the weights of various organs, however, differed considerably. In males, Npc1+/+ and Npc1−/− mice had comparable absolute weights of lungs, spleen, and adrenal glands. In Npc1−/− mice, smaller weights of hearts, livers, kidneys, testes, vesicular, and scent glands were found. In female Npc1−/− mice, ovaries, and uteri were significantly smaller. In Npc1−/− mice, relative organ weights, i.e., normalized with body weights, were sex-specifically altered to different extents by the different therapies. The combination of miglustat, allopregnanolone, and the sterol chelator HPßCD partly normalized the weights of more organs than miglustat or HPßCD mono-therapies.
4
Ko, 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 textAbstract:
People homozygous for mutations in the Niemann-Pick type C1 (NPC1) gene have physiological defects, including excess accumulation of intracellular cholesterol and other lipids, that lead to drastic neural and liver degeneration. The NPC1 multipass transmembrane protein is resident in late endosomes and lysosomes, but its functions are unknown. We find that organelles containing functional NPC1-fluorescent protein fusions undergo dramatic movements, some in association with extending strands of endoplasmic reticulum. InNPC1 mutant cells the NPC1-bearing organelles that normally move at high speed between perinuclear regions and the periphery of the cell are largely absent. Pulse-chase experiments with dialkylindocarbocyanine low-density lipoprotein showed that NPC1 organelles function late in the endocytic pathway; NPC1 protein may aid the partitioning of endocytic and lysosomal compartments. The close connection between NPC1 and the drug U18666A, which causes NPC1-like organelle defects, was established by rescuing drug-treated cells with overproduced NPC1. U18666A inhibits outward movements of NPC1 organelles, trapping membranes and cholesterol in perinuclear organelles similar to those in NPC1 mutant cells, even when cells are grown in lipoprotein-depleted serum. We conclude that NPC1 protein promotes the creation and/or movement of particular late endosomes, which rapidly transport materials to and from the cell periphery.
5
Cawley, 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 textAbstract:
Complex asparagine-linked glycosylation plays key roles in cellular functions, including cellular signaling, protein stability, and immune response. Previously, we characterized the appearance of a complex asparagine-linked glycosylated form of lysosome-associated membrane protein 1 (LAMP1) in the cerebellum of Npc1−/− mice. This LAMP1 form was found on activated microglia, and its appearance correlated both spatially and temporally with cerebellar Purkinje neuron loss. To test the importance of complex asparagine-linked glycosylation in NPC1 pathology, we generated NPC1 knock-out mice deficient in MGAT5, a key Golgi-resident glycosyl transferase involved in complex asparagine-linked glycosylation. Our results show that Mgat5−/−:Npc1−/− mice were smaller than Mgat5+/+:Npc1−/− mice, and exhibited earlier NPC1 disease onset and reduced lifespan. Western blot and lectin binding analyses of cerebellar extracts confirmed the reduction in complex asparagine-linked glycosylation, and the absence of the hyper-glycosylated LAMP1 previously observed. Western blot analysis of cerebellar extracts demonstrated reduced calbindin staining in Mgat5−/−:Npc1−/− mice compared to Mgat5+/+:Npc1−/− mutant mice, and immunofluorescent staining of cerebellar sections indicated decreased levels of Purkinje neurons and increased astrogliosis in Mgat5−/−:Npc1−/− mice. Our results suggest that reduced asparagine-linked glycosylation increases NPC1 disease severity in mice, and leads to the hypothesis that mutations in genes involved in asparagine-linked glycosylation may contribute to disease severity progression in individuals with NPC1. To examine this with respect to MGAT5, we analyzed 111 NPC1 patients for two MGAT5 SNPs associated with multiple sclerosis; however, we did not identify an association with NPC1 phenotypic severity.
6
Dardis, 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 textAbstract:
Niemann-Pick type C (NPC) disease is an autosomal recessive lysosomal storage disorder caused by mutations in NPC1 or NPC2 genes. In 2009, the molecular characterization of 44 NPC Italian patients has been published. Here, we present an update of the genetic findings in 105 Italian NPC patients belonging to 83 unrelated families (77 NPC1 and 6 NPC2). NPC1 and NPC2 genes were studied following an algorithm recently published. Eighty-four different NPC1 and five NPC2 alleles were identified. Only two NPC1 alleles remained non detected. Sixty-two percent of NPC1 alleles were due to missense variants. The most frequent NPC1 mutation was the p.F284Lfs*26 (5.8% of the alleles). All NPC2 mutations were found in the homozygous state, and all but one was severe. Among newly diagnosed patients, 18 novel NPC1 mutations were identified. The pathogenic nature of 7/9 missense alleles and 3/4 intronic variants was confirmed by filipin staining and NPC1 protein analysis or mRNA expression in patient’s fibroblasts. Taken together, our previous published data and new results provide an overall picture of the molecular characteristics of NPC patients diagnosed so far in Italy.
7
Castillo, 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 textAbstract:
A genome-wide association study (GWAS) reported that common variation in the human Niemann-Pick C1 gene ( NPC1) is associated with morbid adult obesity. This study was confirmed using our BALB/cJ Npc1 mouse model, whereby heterozygous mice ( Npc1+/−) with decreased gene dosage were susceptible to weight gain when fed a high-fat diet (HFD) compared with homozygous normal mice ( Npc1+/+) fed the same diet. The objective for our current study was to validate this Npc1 gene-diet interaction using statistical modeling with fitted growth trajectories, conduct body weight analyses for different measures, and define the physiological basis responsible for weight gain. Metabolic phenotype analysis indicated no significant difference between Npc1+/+ and Npc1+/− mice fed a HFD for food and water intake, oxygen consumption, carbon dioxide production, locomotor activity, adaptive thermogenesis, and intestinal lipid absorption. However, the livers from Npc1+/− mice had significantly increased amounts of mature sterol regulatory element-binding protein-1 (SREBP-1) and increased expression of SREBP-1 target genes that regulate glycolysis and lipogenesis with an accumulation of triacylglycerol and cholesterol. Moreover, white adipose tissue from Npc1+/− mice had significantly decreased amounts of phosphorylated hormone-sensitive lipase with decreased triacylglycerol lipolysis. Consistent with these results, cellular energy metabolism studies indicated that Npc1+/− fibroblasts had significantly increased glycolysis and lipogenesis, in addition to significantly decreased substrate (glucose and endogenous fatty acid) oxidative metabolism with an accumulation of triacylglycerol and cholesterol. In conclusion, these studies demonstrate that the Npc1 gene interacts with a HFD to promote weight gain through differential regulation of central energy metabolism pathways.
8
Li, 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 textAbstract:
Export of LDL-derived cholesterol from lysosomes requires the cooperation of the integral membrane protein Niemann–Pick C1 (NPC1) and a soluble protein, Niemann–Pick C2 (NPC2). Mutations in the genes encoding these proteins lead to Niemann–Pick disease type C (NPC). NPC2 binds to NPC1’s second (middle), lumenally oriented domain (MLD) and transfers cholesterol to NPC1’s N-terminal domain (NTD). Here, we report the 2.4-Å resolution crystal structure of a complex of human NPC1–MLD and NPC2 bearing bound cholesterol-3-O-sulfate. NPC1–MLD uses two protruding loops to bind NPC2, analogous to its interaction with the primed Ebola virus glycoprotein. Docking of the NPC1–NPC2 complex onto the full-length NPC1 structure reveals a direct cholesterol transfer tunnel between NPC2 and NTD cholesterol binding pockets, supporting the “hydrophobic hand-off” cholesterol transfer model.
9
Meneses-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 textAbstract:
We recently identified elevated annexin A6 (AnxA6) protein levels in Niemann–Pick-type C1 (NPC1) mutant cells. In these cells, AnxA6 depletion rescued the cholesterol accumulation associated with NPC1 deficiency. Here, we demonstrate that elevated AnxA6 protein levels in NPC1 mutants or upon pharmacological NPC1 inhibition, using U18666A, were not due to upregulated AnxA6 mRNA expression, but caused by defects in AnxA6 protein degradation. Two KFERQ-motifs are believed to target AnxA6 to lysosomes for chaperone-mediated autophagy (CMA), and we hypothesized that the cholesterol accumulation in endolysosomes (LE/Lys) triggered by the NPC1 inhibition could interfere with the CMA pathway. Therefore, AnxA6 protein amounts and cholesterol levels in the LE/Lys (LE-Chol) compartment were analyzed in NPC1 mutant cells ectopically expressing lysosome-associated membrane protein 2A (Lamp2A), which is well known to induce the CMA pathway. Strikingly, AnxA6 protein amounts were strongly decreased and coincided with significantly reduced LE-Chol levels in NPC1 mutant cells upon Lamp2A overexpression. Therefore, these findings suggest Lamp2A-mediated restoration of CMA in NPC1 mutant cells to lower LE-Chol levels with concomitant lysosomal AnxA6 degradation. Collectively, we propose CMA to permit a feedback loop between AnxA6 and cholesterol levels in LE/Lys, encompassing a novel mechanism for regulating cholesterol homeostasis in NPC1 disease.
10
Gläser, 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 textAbstract:
Niemann–Pick type C1 (NPC1) is a lysosomal storage disorder, inherited as an autosomal-recessive trait. Mutations in the Npc1 gene result in malfunction of the NPC1 protein, leading to an accumulation of unesterified cholesterol and glycosphingolipids. Beside visceral symptoms like hepatosplenomegaly, severe neurological symptoms such as ataxia occur. Here, we analyzed the sphingosine-1-phosphate (S1P)/S1P receptor (S1PR) axis in different brain regions of Npc1−/− mice and evaluated specific effects of treatment with 2-hydroxypropyl-β-cyclodextrin (HPβCD) together with the iminosugar miglustat. Using high-performance thin-layer chromatography (HPTLC), mass spectrometry, quantitative real-time PCR (qRT-PCR) and western blot analyses, we studied lipid metabolism in an NPC1 mouse model and human skin fibroblasts. Lipid analyses showed disrupted S1P metabolism in Npc1−/− mice in all brain regions, together with distinct changes in S1pr3/S1PR3 and S1pr5/S1PR5 expression. Brains of Npc1−/− mice showed only weak treatment effects. However, side effects of the treatment were observed in Npc1+/+ mice. The S1P/S1PR axis seems to be involved in NPC1 pathology, showing only weak treatment effects in mouse brain. S1pr expression appears to be affected in human fibroblasts, induced pluripotent stem cells (iPSCs)-derived neural progenitor and neuronal differentiated cells. Nevertheless, treatment-induced side effects make examination of further treatment strategies indispensable.
Dissertations / Theses on the topic "NPC1a"
1
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 textAbstract:
Huntington's disease is an autosomal dominant inherited neurodegenerative disease, with cognitive, motor and psychiatric defects and no known therapy. Altered lipid metabolism and lysosomal dysfunction have been seen in Huntington's disease (HD), including lysosomal expansion, a key feature of a type of disease known as lysosomal storage disorders (LSDs). HD shares similarities with one LSD in particular, Niemann- Pick type C (NPC), suggesting common mechanisms. We have identified NPC-like phenotypes in HD models, including lipid storage, trafficking defects, and lysosomal Ca2+ dysfunction, and the NPC protein NPC1 directly interacts with both normal and mutant huntingtin. We therefore tested the approved NPC therapy miglustat in HD models, and found beneficial effects. Lipid trafficking, Ca2+ signalling and lysosomal storage phenotypes in HD iPSC-derived neuronal cells were improved after miglustat treatment, and climbing ability in Drosophila melanogaster HD models was partially improved. Nucleic acid vectors are an essential tool in HD research and therapy. Lipid- and polymer-based vectors enter the cell via the endocytic system, and may cause altered function. Use of these vectors alone induced lysosomal expansion, phospholipid storage, and altered expression of endocytic proteins, potentially producing artefacts or interfering with delivery of therapies. We have found evidence that HD has lysosomal storage defects which may be due to disruption of NPC1, a previously unidentified component of HD pathology. Our observations also support the theory that huntingtin may be associated with the lysosome under normal as well as pathogenic conditions. The presence of related mechanisms in these two diseases suggests that knowledge from NPC and the LSD field may be useful in HD, and we have demonstrated that the NPC1 therapy miglustat is beneficial in HD models, a finding which will be developed to therapeutic tests in HD patients.
2
Awan, 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 textAbstract:
Nous avons identifié, un ligand de Wnt, Wnt5a, comme faisant partie intégrante du complexe mTORC1, qui régule la fonction lysosomal et favorise le trafic intracellulaire du cholestérol. En diminuant l’activité de mTORC1 et en activant l’axe autophagie-lysosome, Wnt5a adapte les concentration du cholestérol intracellualire aux besoins de la cellule. Wnt5a favorise l’export du cholestérol depuis les endosomal/lysosomal (LELs) vers le réticulum endoplasmique (RE), limite l’accumulation intracellulaire du cholestérol, et protège contre l’athérosclérose. D’un point de vue mécanistique, Wnt5a se lie aux membranes riches en cholestérol et interagit spécifiquement avec la protéine membranaire Niemann-Pick C1 (NPC1), la protéine soluble Niemann-Pick C2 (NPC2), deux protéines lysosomales qui régulent l’export du cholestérol à partir des LELs. En conséquence, l’absence de Wnt5a inhibe la fonction lysosomale et l’autophagie, ainsi que la sortie du cholesterol hors des LELs. Ceci resulte en l’accumulation de larges corps d’inclusion intracellulaires, de larges LELs riches en cholestérol, d’une diminution du cholestérol au niveau du REWe 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
3
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 textAbstract:
In vitro studies have shown that the major membrane phospholipid phosphatidylcholine (PC) can positively influence the incorporation of cholesterol in lipid membranes. The influence of PC on the cellular trafficking of LDL-derived free cholesterol was investigated. Sterol regulatory-defective (SRD)-4 cells are Chinese hamster ovary (CHO)-derived fibroblasts that display vastly elevated rates for the synthesis and catabolism of PC. SRD-4 cells harbor two known gene mutations: a mutation in the functional allele for SCAP, resulting in defective feedback suppression of cholesterol biosynthesis; and a loss-of-function mutation in the functional allele for acyl-CoA:cholesterol acyl transferase (ACAT), an endoplasmic reticulum (ER)-localized enzyme that esterifies free cholesterol. Incubation of SRD-4 cells with 50 µg/ml low density lipoprotein (LDL) for 18 h resulted in lysosomal accumulation of free cholesterol as revealed by filipin staining. This accumulation was not evident following LDL treatment of parental CHO7 cells, and was blunted in SRD-2 cells that express a constitutively-active form of SREBP-2 and overproduce cholesterol but have functional ACAT activity. Treatment of SRD-2 cells with LDL in the presence of an ACAT inhibitor 58-035 resulted in robust lysosomal cholesterol accumulation that was reversible upon drug washout, supporting that cholesterol trafficking in cholesterol-loaded cells is dependent on ACAT activity and, more specifically, ER free cholesterol levels. Lysosomal accumulation of LDL-derived cholesterol was prevented in SRD-4 cells supplemented with lyso-PC (50 µM), a substrate for PC synthesis through the reacylation pathway, and also in cells treated with bromoenol lactone (BEL), an inhibitor of phospholipase A2 implicated in bulk PC turnover. In a counter study, lysosomal LDL-derived cholesterol accumulation was induced in parental CHO-7 cells using R-propranolol, which inhibits the conversion of phosphatidic acid to diacylglycerol (DAG), a substrate in the CDP-choline pathway. This blockage was also relieved through co-treatment with lyso-PC. These studies support that PC to free cholesterol ratios in downstream organellar membranes can influence cholesterol trafficking out of lysosomal compartments in cholesterol-loaded cells.
4
Palladino, 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 textAbstract:
BACKGROUND: The lysosomal storage disorder, Niemann Pick type C1 (NPC1), presents a variable phenotype including neurovisceral and neurological symptoms. 2-Hydroxypropyl-ss-cyclodextrin (HPssCD)-based therapies are presently the most promising route of intervention. While severe cerebellar dysfunction remains the main disabling feature of NPC1, sensory functions including auditory and olfactory ones are also affected. Morphological and functional anomalies of Npc1 (-/-) mouse retina have also been observed, although the functional integrity of the visual pathway from retina to visual cortex is still unsettled. We have addressed this issue by characterizing the visual evoked potential (VEP) response of Npc1 (-/-) mice and determining if/how HPssCD administration influences the VEPs of both Npc1 (-/-) and Npc1 (+/+) mice. METHODS: VEP elicited by a brief visual stimulus were recorded from the scalp overlying the visual cortex of adult (PN, postnatal days 60, 75, 85 and 100) Npc1 (+/+) and Npc1 (-/-) mice that had received repeated injections of either HPssCD or plain vehicle. The first injection was given at PN4 and was followed by a second one at PN7 and thereafter by weekly injections up to PN49. Cholesterol accumulation and myelin loss were finally assessed by filipin staining and myelin basic protein immunohistochemistry, respectively. RESULTS AND DISCUSSION: We have found that the transmission of visual signals from retina to visual cortex is negatively influenced by the loss of Npc1 function. In fact, the VEP response of Npc1 (-/-) mice displayed a highly significant increase in the latency compared to that of Npc1 (+/+) mice. HPssCD administration fully rescued this defect and counteracted the cholesterol accumulation in retinal ganglion cells and dorsal lateral geniculate nucleus neurons, as well as the myelin loss in optic nerve fibers and axons projecting to the visual cortex observed in of Npc1 (-/-) mice. By contrast, HPssCD administration had no effect on the VEP response of Npc1 (+/+) mice, further strengthening the treatment efficacy. CONCLUSIONS: This study pinpoints the analysis of VEP response as a potentially accurate and non-invasive approach to assess neural activity and visual information processing in NPC1 patients, as well as for monitoring the progression of the disease and assessing the efficacy of potential therapies.
5
Almeida, 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 textAbstract:
Niemann-Pick tipo C (NPC) é uma doença de depósito lisossomal, ocasionada por alterações no tráfico de colesterol não esterificado, decorrente de alterações bialélicas nos genes NPC1 ou NPC2, ambos definindo uma doença autossômica recessiva, progressiva e irreversível, caracterizada por manifestações viscerais, neurológicas e psiquiátricas, não necessariamente combinadas. Com o propósito de descrever as características fenotípicas e genotípicas de pacientes com NPC, objetivou-se relatar dados demográficos, formas clínicas classificadas por idade, sinais e sintomas neurológicos e psiquiátricos, achados de ressonância magnética (RM) de encéfalo e ultrassonografia de abdome, assim como teste de filipin, mutações observadas e o tratamento com N-butyldeoxynojirimycin (Miglustat). De uma casuística de 12 pacientes atendidos entre 2000-2014, por revisão de prontuários, no Ambulatório de Neurogenética do Hospital das Clínicas da Faculdade de Medicina de Ribeirão Preto, havia 7 mulheres e 5 homens, idade média de 20 anos (entre 2 e 42), sendo dez pacientes da etnia branca e dois mulatos, procedentes de 3 estados brasileiros: São Paulo, Mato Grosso do Sul e Minas Gerais. As formas clínicas identificadas foram infantil, juvenil e adulto. A idade do primeiro sintoma neurológico ocorreu entre 1 e 27 anos (media 9,5). Dentre os achados viscerais, dois pacientes encontravam-se assintomáticos, os demais apresentaram icterícia prolongada/colestase, hepatomegalia e esplenomegalia. Todos os pacientes apresentaram em diferentes momentos da evolução manifestações neuropsiquiátricas, tais como: paralisia do olhar vertical, ataxia/quedas, epilepsia, mioclonias, distonia, disartria, disfagia, fraqueza muscular, espasticidade, declínio cognitivo/demência, sintomas psicóticos, atraso escolar, distúrbio de comportamento, cataplexia gelástica e hipotonia neonatal. A idade de diagnóstico variou de 0 a 41 anos, com uma média de 14,5 anos. O tempo entre a idade do primeiro sintoma neurológico e o diagnóstico da doença variou de 0 a 14 anos, tempo médio de 5,3 anos. O teste de Filipin demonstrou seis resultados positivos e seis variantes. A RM de encéfalo apresentou três diferentes tipos de alteração: atrofia cerebral em 6 casos, atrofia cerebelar e desmielinização em 7. A ultrassonografia de Abdome resultou em três alterações: hepatomegalia em 8, esplenomegalia em 10 e hepatoesplenomegalia em 8. O resultado do teste genético molecular em 11 pacientes evidenciou alterações no gene NPC1 e uma paciente não possuía o resultado. A mutação c.3104C>T foi a mais frequente, em oito pacientes; c.3548G>A, de significado incerto, em um paciente, e, as demais mutações encontradas: c.3493G>A, c.3019C>G. O tratamento com N-butyl deoxynojirimycin (Miglustat) foi realizado por todos os pacientes, o tempo entre o diagnóstico e o início da medicação variou de 0 a 9 anos, média de 2,9 anos. Concluímos que o registro da doença NPC deve ser feito através de uma coleta de dados detalhada e contínua, pois sua heterogeneidade fenotípica e genotípica sugerem um número subestimado de casos, não só por sua raridade, mas também pelo desconhecimento da doença, já que há poucos grupos estudados e publicados. O seu reconhecimento precoce, associado ao adequado manejo clínico, podem retardar a progressão implacável da doença e aumentar a expectativa de vida dos pacientes.Niemann-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.
6
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 textAbstract:
Ebolavirus (EboV) is a highly pathogenic enveloped virus that causes outbreaks of zoonotic infection in Africa. The clinical symptoms are manifestations of the massive production of pro-inflammatory cytokines in response to infection and in many outbreaks, case fatality rate exceeds 75%. The unpredictable onset, ease of transmission, rapid progression of disease, high mortality and lack of effective vaccine or therapy have created a high level of public concern about EboV. Here we report the properties of a benzylpiperazine adamantane diamide-derived compound identified in a screen for inhibitors of EboV infection. We found that the inhibitor is specific, reversible, and that the target(s) for inhibition are present in cells and not in virus particles. The compound is not an inhibitor of acid pH-dependent endosome protease activity, which is required for EboV infection. Treatment of cells with this compound causes accumulation of cholesterol in late endosomes and lysosomes (LE/LY), suggesting it inhibits one or more proteins involved in regulation of cholesterol uptake into cells. Using mutant cell lines and informative derivatives of the inhibitor, we found the inhibitor target is the endosomal membrane protein Niemann-Pick C1 (NPC1). NPC1 is a polytopic LE/LY membrane protein that mediates uptake of lipoprotein-derived cholesterol into cells. We find that NPC1 is essential for EboV infection, that NPC1 binds to the protease-cleaved GP1 subunit of the EboV glycoprotein, and that the anti-viral compound inhibits infection by targeting NPC1 and interfering with binding to GP1. Furthermore, analysis of viral variants resistant to the anti-viral compound revealed that the residues which confer resistance are located on the surface of the receptor binding domain of GP1. Combined with the results of previous studies of GP structure and function, our findings support a model of EboV infection in which cleavage of the GP1 subunit by endosomal cathepsin proteases removes heavily glycosylated domains to expose the N-terminal domain, which is a ligand for NPC1 and regulates membrane fusion by the GP2 subunit. Thus, NPC1 is essential for EboV entry and a target for anti-viral therapy.
7
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/18483.
Full textAbstract:
Дисертація на здобуття наукового ступеня кандидата технічних наук за спеціальністю 05.17.01 – технологія неорганічних речовин. – Національний технічний університет "Харківський політехнічний інститут", Харків, 2015. Дисертацію присвячено розробці наукових основ технології комплексних NPCa-добрив з мікроелементами із залученням фосфоровмісної сировини з низьким вмістом фосфору (V) оксиду. Вперше на основі термодинамічних розрахунків, експериментальних досліджень та рентгенофазових аналізів визначено перелік реакцій, що перебігають в процесі взаємодії карбаміду з нітратно-кислотною витяжкою (НКВ). Визначено технологічні параметри взаємодії карбаміду з НКВ та процесу амонізації NPCа-добрив, а також розроблено принципову схему, яка дозволяє отримати низку комплексних NPCa-добрив з мікроелементами по безвідходній технології із залученням фосфоровмісної сировини України з низьким вмістом фосфору (V) оксиду. Встановлено, що взаємодія карбаміду з НКВ відбувається у кінетичній області та створено кінетичну модель процесу, знайдено константи швидкості, енергію активації. Створена математична модель, яка дозволяє розрахувати технологічні параметри процесу і хімічний реактор синтезу. Отримане комплексне NPCa-добриво з мікроелементами завдяки високому вмісту водорозчинної та засвоюваної форм Р₂О₅ та СаО при агрохімічних випробуваннях у Національному науковому центрі "Інститут ґрунтознавства і агрохімії імені О. Н. Соколовського" показало на підвищення врожайності овочевих культур до 12 ÷ 30 %. Доведено їх економічну доцільність.Dissertation 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.
8
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 textAbstract:
L’endosymbiose trophique établie entre un hôte Cnidaire et ses symbiotes Dinoflagellés photosynthétiques est à l’origine du succès évolutif des écosystèmes coralliens. Les symbiotes sont internalisés par un mécanisme de phagocytose et maintenus dans les cellules du gastroderme de l'hôte. La symbiose est régie par un dialogue moléculaire intime entre les deux partenaires, interrompu lors de perturbations environnementales ou anthropiques, responsables du déclin mondial des récifs coralliens. Les objectifs de mon projet de recherche sont de définir les acteurs moléculaires localisés à l’interface symbiotique chez l’anémone de mer, Anemonia viridis. Premièrement, nous avons étudié les mécanismes cellulaires impliqués dans différents types de rupture de la symbiose et mis en évidence des phénomènes d’apoptose, nécrose et symbiophagie. Parallèlement, nous avons caractérisé chez l’anémone les gènes npc1 et npc2, impliqués chez les vertébrés dans le transport endosomal de stérols, et dont l’expression est modulée par l’état symbiotique. Nous avons pu montrer que le gène npc2d est issus d’une duplication et vraisemblablement d’une sub-fonctionnalisation et que les protéines NPC1 et NPC2 sont exprimées au voisinage des symbiotes. Nous proposons donc que la protéine NPC2-d soit utilisée comme marqueur de l’état de santé des Anthozoaires symbiotiques et que la protéine NPC1 soit un marqueur de la membrane périsymbiotique. Nous avons également développé un protocole afin d’identifier les protéines associées à l’interface symbiotique entre les deux partenaires. A terme, les cibles identifiées permettront une meilleure compréhension des mécanismes qui régulent la relation symbiotiqueThe 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
9
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 textAbstract:
Дисертація на здобуття наукового ступеня кандидата технічних наук за спеціальністю 05.17.01 – технологія неорганічних речовин. – Національний технічний університет "Харківський політехнічний інститут", Харків, 2015.
Дисертацію присвячено розробці наукових основ технології комплексних NPCa-добрив з мікроелементами із залученням фосфоровмісної сировини з низьким вмістом фосфору (V) оксиду. Вперше на основі термодинамічних розрахунків, експериментальних досліджень та рентгенофазових аналізів визначено перелік реакцій, що перебігають в процесі взаємодії карбаміду з нітратно-кислотною витяжкою (НКВ). Визначено технологічні параметри взаємодії карбаміду з НКВ та процесу амонізації NPCа-добрив, а також розроблено принципову схему, яка дозволяє отримати низку комплексних NPCa-добрив з мікроелементами по безвідходній технології із залученням фосфоровмісної сировини України з низьким вмістом фосфору (V) оксиду. Встановлено, що взаємодія карбаміду з НКВ відбувається у кінетичній області та створено кінетичну модель процесу, знайдено константи швидкості, енергію активації. Створена математична модель, яка дозволяє розрахувати технологічні параметри процесу і хімічний реактор синтезу. Отримане комплексне NPCa-добриво з мікроелементами завдяки високому вмісту водорозчинної та засвоюваної форм Р₂О₅ та СаО при агрохімічних випробуваннях у Національному науковому центрі "Інститут ґрунтознавства і агрохімії імені О. Н. Соколовського" показало на підвищення врожайності овочевих культур до 12 ÷ 30 %. Доведено їх економічну доцільність.Dissertation 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.
10
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 textAbstract:
The studies in this thesis describe the application of genotyping and allele specific expression analysis to genetic studies. The role of the gene NPC1 in Triglyceride metabolism was explored in mouse models and in humans on the population level in study I. NPC1 was found to affect hepatic triglyceride metabolism, and to be relevant for controlling serum triglyceride levels in mice and potentially in humans. In study II the utility of the HapMap CEU samples was investigated for tagSNP selection in six European populations. The HapMap CEU was found to be representative for tagSNP selection in all populations while allele frequencies differed significantly in the sample from Kuusamo, Finland. In study III the power of Allele specific expression as a tool for the mapping of cis-regulatory variation was compared to standard eQTL analysis, ASE was found to be the more powerful type of analysis for a similar sample size. Finally ASE mapping was applied to regions reported to harbour long non-coding RNAs and associated SNPs were compared to published trait-associations. This revealed strong cis-regulatory SNPs of long non-coding RNAs with reported trait or disease associations.
Books on the topic "NPC1a"
1
Smith, Eric H. NPCA field guide to structural pests. [Dunn Loring, Va.]: NPCA, 1992.
Find full text
2
Inc, 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 text
3
Sedel, Frédéric. Niemann-Pick Disease Type C. Oxford University Press, 2016. http://dx.doi.org/10.1093/med/9780199972135.003.0053.
Full textAbstract:
Niemann-Pick disease type C (NPC) is a fatal neurovisceral lipid storage disease of autosomal inheritance resulting from mutations in either the NPC1 (95% of families) or NPC2 gene. The encoded proteins appear to be involved in lysosomal/late endosomal transport of cholesterol, glycolipids, and other molecules, but their exact function is still unknown. The clinical spectrum of the disease ranges from a neonatal rapidly fatal disorder to an adult-onset chronic neurodegenerative disease characterized prominently by psychiatric disorders, cerebellar ataxia, cognitive decline, and vertical supranuclear gaze palsy. Miglustat is the only treatment approved to date which has been demonstrated to slow or halt disease progression.
4
National 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"
1
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 text
2
Miles, 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 text
3
Cologna, 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 text
4
Zampieri, 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
5
"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 text
6
Turner-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"
1
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 text
2
Ying, 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 text
3
Xin 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 text
4
Hao, 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 text
5
Slawinski, 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.
Full text