A new regulatory mechanism of STARD1 in Niemann-Pick disease type C (NPC), discovered
Por um escritor misterioso
Last updated 19 setembro 2024
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 C1 is a sphingosine storage disease that causes deregulation of lysosomal calcium
Frontiers Intracellular Cholesterol Trafficking and Impact in Neurodegeneration
Niemann‐Pick type C disease: molecular mechanisms and potential therapeutic approaches - Rosenbaum - 2011 - Journal of Neurochemistry - Wiley Online Library
Proposed pathological cascade in Niemann-Pick disease type C (NPC)
Mitochondrial GSH replenishment as a potential therapeutic approach for Niemann Pick type C disease - ScienceDirect
STARD1 and NPC1 expression as pathological markers associated with astrogliosis in post-mortem brains from patients with Alzheimer's disease and Down syndrome
Niemann-Pick C disease and mobilization of lysosomal cholesterol by cyclodextrin - ScienceDirect
Lysosomal positioning diseases: beyond substrate storage
The Steroidogenic Acute Regulatory Protein (StAR)
Acid ceramidase improves mitochondrial function and oxidative stress in Niemann-Pick type C disease by repressing STARD1 expression and mitochondrial cholesterol accumulation - ScienceDirect
Mitochondrial GSH replenishment as a potential therapeutic approach for Niemann Pick type C disease - ScienceDirect
Niemann-Pick type C disease: The atypical sphingolipidosis. - Abstract - Europe PMC
Sterol regulation of the expression and transcription of the
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