[HTML][HTML] Altered cholesterol metabolism in Niemann-Pick type C1 mouse brains affects mitochondrial function
W Yu, JS Gong, M Ko, WS Garver… - Journal of biological …, 2005 - ASBMB
Niemann-Pick type C1 (NPC1) disease is a fatal hereditary disorder characterized by a
defect in cholesterol trafficking and progressive neurodegeneration. Although the NPC1
gene has been identified, the molecular mechanism responsible for neuronal dysfunction in
brains of patients with NPC1 disease remains unknown. This study demonstrates that the
amount of cholesterol within mitochondria membranes is significantly elevated in NPC1
mouse brains and neural cells. In addition, the mitochondrial membrane potential, the …
defect in cholesterol trafficking and progressive neurodegeneration. Although the NPC1
gene has been identified, the molecular mechanism responsible for neuronal dysfunction in
brains of patients with NPC1 disease remains unknown. This study demonstrates that the
amount of cholesterol within mitochondria membranes is significantly elevated in NPC1
mouse brains and neural cells. In addition, the mitochondrial membrane potential, the …