Low-density lipoprotein particles are taken up by cells and delivered to the lysosome where
their cholesterol esters are cleaved off by acid lipase. The released, free cholesterol is then …

Cholesterol is an important precursor for numerous biologically active molecules, and it
plays a major role in membrane structure and function. Cholesterol can be endogenously …

Niemann-Pick disease type C (NPC) is characterized by lysosomal storage of cholesterol
and gangliosides, which results from defects in intracellular lipid trafficking. Most studies of …

Cells acquire cholesterol either by de novo synthesis in the endoplasmic reticulum or by
internalization of cholesterol-containing lipoproteins, particularly low density lipoprotein …

Niemann-Pick type C (NPC) disease is an inherited lipid storage disorder that affects the
viscera and central nervous system. A characteristic feature of NPC cells is the lysosomal …

Niemann-Pick disease type C (NPC) is caused by defects in either the NPC1 or NPC2 gene
and is characterized by accumulation of cholesterol and glycolipids in the late …

Summary Water-soluble Niemann-Pick C2 (NPC2) and membrane-bound NPC1 are
cholesterol-binding lysosomal proteins required for export of lipoprotein-derived cholesterol …

Lysosomal cholesterol egress requires two proteins, NPC1 and NPC2, whose defects are
responsible for Niemann-Pick disease type C (NPC). Here, we present systematic structural …

The Niemann–Pick C protein (NPC1) is required for cholesterol transport from late
endosomes and lysosomes to other cellular membranes. Mutations in NPC1 cause …

Niemann–Pick type C1 (NPC1) protein is needed for cellular utilization of low-density
lipoprotein-derived cholesterol that has been delivered to lysosomes. The protein has 13 …