NPC1 - Niemann-Pick C1 protein - human protein (Function)
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NPC1 »  Niemann-Pick C1 protein
Gene name:  NPC1
Family name: Patched
Entry whose protein(s) existence is based on evidence at protein level
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Intracellular cholesterol transporter which acts in concert with NPC2 and plays an important role in the egress of cholesterol from the endosomal/lysosomal compartment. Both NPC1 and NPC2 function as the cellular 'tag team duo' (TTD) to catalyze the mobilization of cholesterol within the multivesicular environment of the late endosome (LE) to effect egress through the limiting bilayer of the LE. NPC2 binds unesterified cholesterol that has been released from LDLs in the lumen of the late endosomes/lysosomes and transfers it to the cholesterol-binding pocket of the N-terminal domain of NPC1. Cholesterol binds to NPC1 with the hydroxyl group buried in the binding pocket and is exported from the limiting membrane of late endosomes/ lysosomes to the ER and plasma membrane by an unknown mechanism. Binds oxysterol with higher affinity than cholesterol. May play a role in vesicular trafficking in glia, a process that may be crucial for maintaining the structural and functional integrity of nerve terminals.  
  • CuratedUniProtKB
According to TCDB this is a transporter from family:
the resistance-nodulation-cell division (rnd) superfamily  2.A.6.6.1  
According to KEGG, this protein belongs to the following pathway:
Lysosome  hsa04142+4864  


Biological process 
Cholesterol metabolism  definition   [KW-0153]
Lipid metabolism  definition   [KW-0443]
Steroid metabolism  definition   [KW-0753]
Sterol metabolism  definition   [KW-1207]
Technical term 
Reference proteome  definition   [KW-1185]

Further external links

GeneWiki: NPC1
GenomeRNAi: 4864
PRO: PR:O15118