NPC1L1

NPC1 like intracellular cholesterol transporter 1

Summary

The protein encoded by this gene is a multi-pass membrane protein. It contains a conserved N-terminal Niemann-Pick C1 (NPC1) domain and a putative sterol-sensing domain (SSD) which includes a YQRL motif functioning as a plasma membrane to trans-Golgi network transport signal in other proteins. This protein takes up free cholesterol into cells through vesicular endocytosis and plays a critical role in the absorption of intestinal cholesterol. It also has the ability to transport alpha-tocopherol (vitamin E). The drug ezetimibe targets this protein and inhibits the absorption of intestinal cholesterol and alpha-tocopherol. In addition, this protein may play a critical role in regulating lipid metabolism. Polymorphic variations in this gene are associated with plasma total cholesterol and low-density lipoprotein cholesterol (LDL-C) levels and coronary heart disease (CHD) risk. Alternatively spliced transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Oct 2009]

Known Variants123 total

rsidPosition (GRCh37)AllelesClassClinVar
rs7515721897:44,553,056C/Tlikely benign
rs7550849667:44,553,057G/Auncertain significance
rs7750252377:44,553,112A/Cuncertain significance
rs2174357:44,553,204C/Tbenign
rs1473779457:44,553,233G/Auncertain significance
rs2174347:44,553,238A/Gsynonymous variantbenign
rs2011875897:44,553,240C/Tlikely benign
rs102647157:44,555,406G/Abenign
rs1152815907:44,555,421G/Alikely benign
rs7606563597:44,555,468G/Cuncertain significance
rs1485065067:44,555,510C/Auncertain significance
rs2174287:44,555,573T/Gregulatory region variantbenign
rs528150637:44,555,699A/Tmissense variantdrug response
rs5283521147:44,556,303G/Auncertain significance
rs8959734767:44,556,367T/Cuncertain significance
rs5524988407:44,556,475G/Tuncertain significance
rs1167161607:44,556,509C/Glikely benign
rs7781817347:44,556,832G/Tuncertain significance
rs795197447:44,556,834C/Auncertain significance
rs7676264557:44,556,890G/Cuncertain significance
rs1153956827:44,556,893C/Tbenign
rs77995947:44,558,217G/Auncertain significance
rs13162775257:44,558,224T/Cuncertain significance
rs14808109507:44,558,247G/Auncertain significance
rs8792533927:44,558,260C/Tuncertain significance
rs7508119607:44,558,261T/Clikely benign
rs7632606287:44,560,399G/Auncertain significance
rs10604998857:44,560,414A/Tuncertain significance
rs5392090497:44,560,419G/Alikely benign
rs1475198517:44,560,582C/Tbenign
rs13500934427:44,561,313A/Tuncertain significance
rs1497829077:44,561,344G/Abenign
rs7474140357:44,561,355T/Auncertain significance
rs7672784887:44,561,370G/Auncertain significance
rs1508913927:44,561,702T/Cuncertain significance
rs24842958467:44,561,794C/Guncertain significance
rs1142398197:44,561,806G/Abenign
rs47204707:44,561,884C/Tintron variantdrug response
rs117637597:44,570,067T/Cintron variantdrug response
rs7656855207:44,571,364G/Tuncertain significance
rs23019357:44,571,460T/A
rs1436810667:44,571,712G/Clikely benign
rs1143742797:44,571,721A/Gbenign
rs1490852357:44,571,741G/Auncertain significance
rs5646923387:44,571,756C/Tuncertain significance
rs1133774327:44,571,763A/Cbenign
rs1512114287:44,571,767G/Alikely benign
rs7515282757:44,571,779T/Clikely benign
rs7482720807:44,571,810G/Auncertain significance
rs13402699597:44,573,407G/Cuncertain significance
rs7549602047:44,573,412A/Guncertain significance
rs7578283927:44,574,066T/Cuncertain significance
rs7708979667:44,574,134C/Tuncertain significance
rs24843363797:44,574,155A/Cuncertain significance
rs24843366647:44,574,191G/Auncertain significance
rs3735605737:44,574,215G/Auncertain significance
rs24843369307:44,574,216C/Tuncertain significance
rs1154647787:44,575,487C/Tlikely benign
rs7503853907:44,575,500A/Guncertain significance
rs3730917107:44,575,563G/Auncertain significance
rs11838150177:44,575,566C/Auncertain significance
rs1455542417:44,575,863T/Cuncertain significance
rs2007532607:44,575,892C/Tuncertain significance
rs2021272867:44,575,896G/Auncertain significance
rs5365399297:44,575,920A/Cuncertain significance
rs3775146727:44,575,956G/Auncertain significance
rs1430824017:44,575,987G/Alikely benign
rs2003366227:44,576,021T/Cuncertain significance
rs10018399097:44,576,446T/Cuncertain significance
rs13058084447:44,576,477G/Tuncertain significance
rs24843481337:44,576,509A/Guncertain significance
rs13767036497:44,578,431A/Cuncertain significance
rs358031017:44,578,500G/Amissense variant
rs795730117:44,578,503C/Tuncertain significance
rs2014215147:44,578,579C/Tuncertain significance
rs10430565927:44,578,581T/Cuncertain significance
rs3775615887:44,578,588T/Cuncertain significance
rs1485412537:44,578,622C/Tlikely benign
rs11816298077:44,578,740C/Auncertain significance
rs5366601317:44,578,746C/Tuncertain significance
rs1396596537:44,578,747G/Alikely benign
rs1452977997:44,578,780G/Astop gainedassociation
rs7464723847:44,578,783A/Guncertain significance
rs5493572987:44,578,870T/Auncertain significance
rs1488598057:44,578,885C/Auncertain significance
rs2000728787:44,578,893G/Auncertain significance
rs2005848767:44,578,911G/Alikely benign
rs7608376097:44,578,917A/Guncertain significance
rs1434944047:44,578,918C/Tbenign
rs1483997877:44,578,937C/Glikely benign
rs5321386077:44,578,958C/Tlikely benign
rs13406085107:44,578,959G/Auncertain significance
rs24843588617:44,579,014T/Cuncertain significance
rs1998027417:44,579,017C/Tuncertain significance
rs1451672957:44,579,062C/Guncertain significance
rs798037007:44,579,068C/Abenign
rs3729133707:44,579,150C/Tlikely benign
rs5704099617:44,579,171G/Alikely benign
rs1381402507:44,579,179C/Abenign
rs20721837:44,579,180G/Csynonymous variantdrug response

Showing 100 of 123 variants. Use the SNP search for the full list.

Gene information from NCBI Gene. Variant classifications from ClinVar.