Spreading the wealth: Niemann-Pick type C proteins bind and transport cholesterol.
Endocytosed cholesterol must be transferred from the environment (e.g., low-density lipoproteins) via the lysosomal system to the rest of the cell. In Niemann-Pick type C disease, this process fails. In a recent issue of Cell, Kwon et al. (2009) suggest how this transpires mechanistically by crystallizing a domain of a protein defective in this syndrome.
Munkacsi
Andrew B
AB
Department of Pediatrics, Columbia University Medical Center, New York, NY 10032, USA.
Pentchev
Peter G
PG
Sturley
Stephen L
SL
D001692:Biological Transport; D002352:Carrier Proteins / Q000737:chemistry / Q000378:metabolism*; D002784:Cholesterol / Q000378:metabolism*; D006801:Humans; D047908:Intracellular Signaling Peptides and Proteins; D008562:Membrane Glycoproteins / Q000737:chemistry / Q000378:metabolism*; D000077296:Niemann-Pick C1 Protein; D052556:Niemann-Pick Disease, Type C / Q000378:metabolism; D011485:Protein Binding; D017434:Protein Structure, Tertiary
D002352:Carrier Proteins; D047908:Intracellular Signaling Peptides and Proteins; D008562:Membrane Glycoproteins; C106881:NPC1 protein, human; D000077296:Niemann-Pick C1 Protein; D002784:Cholesterol