Journal Club

NPC1-mTORC1 Signaling Couples Cholesterol Sensing to Organelle Homeostasis and Is a Targetable Pathway in Niemann-Pick Type C

Developmental Cell VOLUME 56, ISSUE 3, P260-276.E7, FEBRUARY 08, 2021

Highlights

• Proteomic profiling of NPC lysosomes reveals both proteolytic and structural defects

• Loss of cholesterol transport activity by NPC1 causes aberrant mTORC1 signaling

• mTORC1 inhibition restores lysosomal and mitochondrial function in NPC cells