A new regulatory mechanism of STARD1 in Niemann-Pick disease type C (NPC), discovered

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.

Critical role for glycosphingolipids in Niemann-Pick disease type C: Current Biology

Early steps in steroidogenesis: intracellular cholesterol trafficking. - Abstract - Europe PMC

Mitochondrial dysfunction in fibroblasts derived from patients with Niemann-Pick type C disease - ScienceDirect

Mitochondrial cholesterol trafficking and StARD1 expression in human

Role of STARD4 and NPC1 in intracellular sterol transport

Niemann-Pick disease type C

The Steroidogenic Acute Regulatory Protein (StAR)

Niemann-Pick Type C Disease Reveals a Link between Lysosomal Cholesterol and PtdIns(4,5)P2 That Regulates Neuronal Excitability - ScienceDirect

NPC1 enables cholesterol mobilization during long‐term potentiation that can be restored in Niemann–Pick disease type C by CYP46A1 activation

Niemann-Pick C disease and mobilization of lysosomal cholesterol by cyclodextrin - ScienceDirect

Biomolecules, Free Full-Text

Mitochondrial function and regulation of macrophage sterol metabolism and inflammatory responses

Frontiers The role of cholesterol and mitochondrial bioenergetics in activation of the inflammasome in IBD

A new regulatory mechanism of STARD1 in Niemann-Pick disease type C (NPC), discovered

UPR analysis in NPC mice cerebellum. a NPC and wildtype (WT) mice were