A new regulatory mechanism of STARD1 in Niemann-Pick disease type C (NPC), discovered
Descrição
Researchers from the group Mitochondrial regulation of cell death and steatohepatitis have revealed a new role for acid ceramidase (ACDase) in experimental models and in patients with NPC through the regulation of STARD1 and its impact on mitochondrial function.
Niemann-Pick disease type C
Frontiers Intracellular Cholesterol Trafficking and Impact in Neurodegeneration
Niemann-Pick type C disease: The atypical sphingolipidosis. - Abstract - Europe PMC
Mitochondrial dysfunction in fibroblasts derived from patients with Niemann-Pick type C disease - ScienceDirect
Niemann-Pick C disease and mobilization of lysosomal cholesterol by cyclodextrin - ScienceDirect
Genetic and Chemical Correction of Cholesterol Accumulation and Impaired Autophagy in Hepatic and Neural Cells Derived from Niemann-Pick Type C Patient-Specific iPS Cells - ScienceDirect
The pathogenesis of Niemann–Pick type C disease: a role for autophagy?, Expert Reviews in Molecular Medicine
IJMS, Free Full-Text
Mitochondrial GSH replenishment as a potential therapeutic approach for Niemann Pick type C disease - ScienceDirect
Niemann-Pick disease type C1 is a sphingosine storage disease that causes deregulation of lysosomal calcium
NPC1 - Wikipedia
Novel Mutation in the Feline NPC2 Gene in Cats with Niemann–Pick Disease
NPC1 - Wikipedia
Cells, Free Full-Text
UPR analysis in NPC mice cerebellum. a NPC and wildtype (WT) mice were
de
por adulto (o preço varia de acordo com o tamanho do grupo)