AMACR

alpha-methylacyl-CoA racemase

Summary

This gene encodes a racemase. The encoded enzyme interconverts pristanoyl-CoA and C27-bile acylCoAs between their (R)- and (S)-stereoisomers. The conversion to the (S)-stereoisomers is necessary for degradation of these substrates by peroxisomal beta-oxidation. Encoded proteins from this locus localize to both mitochondria and peroxisomes. Mutations in this gene may be associated with adult-onset sensorimotor neuropathy, pigmentary retinopathy, and adrenomyeloneuropathy due to defects in bile acid synthesis. Alternatively spliced transcript variants have been described. Read-through transcription also exists between this gene and the upstream neighboring C1QTNF3 (C1q and tumor necrosis factor related protein 3) gene. [provided by RefSeq, Mar 2011]

Known Variants344 total

rsidPosition (GRCh37)AllelesClassClinVar
rs68676415:33,985,857T/C
rs132895:33,986,409C/T
rs8860605215:33,987,105T/Cuncertain significance
rs1823619975:33,987,110T/Auncertain significance
rs5291403465:33,987,154G/Tuncertain significance
rs9714696055:33,987,213C/Tuncertain significance
rs798804515:33,987,253T/Clikely benign
rs5437258875:33,987,256G/Auncertain significance
rs5633328545:33,987,267C/Guncertain significance
rs5319921285:33,987,270A/Glikely benign
rs10062306295:33,987,323G/Auncertain significance
rs10167744065:33,987,336G/Auncertain significance
rs8860605225:33,987,345C/Tuncertain significance
rs5630652715:33,987,346G/Abenign
rs1162065025:33,987,394C/Alikely benign
rs13837453865:33,987,434T/Guncertain significance
rs1163338335:33,987,471C/Abenign
rs5371618885:33,987,555A/Guncertain significance
rs8860605235:33,987,576A/Guncertain significance
rs8860605245:33,987,638C/Tuncertain significance
rs7524737895:33,987,831A/Guncertain significance
rs5305071955:33,987,832T/Clikely benign
rs8860605255:33,987,845C/Tuncertain significance
rs1903292715:33,987,995T/Clikely benign
rs8860605265:33,988,017C/Tuncertain significance
rs8860605275:33,988,020C/Tuncertain significance
rs1419694655:33,988,088C/Auncertain significance
rs5508911725:33,988,166C/Guncertain significance
rs1506640865:33,988,222C/Tlikely benign
rs3675827575:33,988,393G/Tuncertain significance
rs12874776565:33,988,424T/Cuncertain significance
rs9910529895:33,988,438G/Auncertain significance
rs1400215995:33,988,445A/Cbenign
rs1438774675:33,988,484A/Gconflicting classifications of pathogenicity
rs1463852385:33,988,658A/Guncertain significance
rs5752646855:33,988,675G/Auncertain significance
rs5505023565:33,988,749A/Glikely benign
rs5330949755:33,988,759T/Cuncertain significance
rs5572945155:33,988,965A/Guncertain significance
rs11825232875:33,989,198T/Guncertain significance
rs24789505495:33,989,207A/Glikely benign
rs14843351385:33,989,210T/Auncertain significance
rs24789506225:33,989,221T/Auncertain significance
rs2002708225:33,989,224T/Cuncertain significance
rs24789506625:33,989,228A/Glikely benign
rs17533882205:33,989,233T/Cuncertain significance
rs21120320285:33,989,238T/Cuncertain significance
rs24789508135:33,989,239C/Guncertain significance
rs7737802975:33,989,250T/Cuncertain significance
rs7744064485:33,989,263C/Tuncertain significance
rs5616754075:33,989,264G/Aconflicting classifications of pathogenicity
rs3746682565:33,989,265C/Tuncertain significance
rs15613797505:33,989,266G/Auncertain significance
rs1380525405:33,989,277A/Guncertain significance
rs7614885825:33,989,296C/Tuncertain significance
rs7797229355:33,989,302G/Cuncertain significance
rs21120322375:33,989,311T/Cuncertain significance
rs11893291515:33,989,315A/Tuncertain significance
rs24789515015:33,989,320C/Auncertain significance
rs17533947865:33,989,321C/Tlikely benign
rs7497036995:33,989,322C/Guncertain significance
rs11600111945:33,989,330A/Glikely benign
rs7792904955:33,989,336G/Aconflicting classifications of pathogenicity
rs7722196695:33,989,344G/Auncertain significance
rs14762132975:33,989,345G/Alikely benign
rs3686937385:33,989,346G/Auncertain significance
rs12657146655:33,989,353A/Cuncertain significance
rs7607271615:33,989,354C/Tlikely benign
rs7617683315:33,989,363T/Clikely benign
rs24789518325:33,989,364G/Auncertain significance
rs7649619915:33,989,369G/Clikely benign
rs3713433455:33,989,371G/Auncertain significance
rs7661165095:33,989,372G/Alikely benign
rs2005058395:33,989,380C/Tconflicting classifications of pathogenicity
rs9067978725:33,989,381G/Alikely benign
rs5752632045:33,989,382T/Auncertain significance
rs24789520885:33,989,386G/Cuncertain significance
rs21120325765:33,989,397G/Tuncertain significance
rs24789521945:33,989,402A/Cuncertain significance
rs1133616295:33,989,403A/Tuncertain significance
rs1399730155:33,989,405C/Tlikely benign
rs7471295415:33,989,406G/Auncertain significance
rs7687523425:33,989,408G/Alikely benign
rs8860605295:33,989,410C/Tuncertain significance
rs2015071695:33,989,413G/Auncertain significance
rs12348928265:33,989,433T/Auncertain significance
rs7729463505:33,989,434G/Auncertain significance
rs7628203815:33,989,437C/Auncertain significance
rs24789526465:33,989,448A/Guncertain significance
rs8860605305:33,989,458C/Tuncertain significance
rs9746237185:33,989,459C/Tlikely benign
rs7741803975:33,989,460G/Auncertain significance
rs21120327925:33,989,463G/Auncertain significance
rs12715581365:33,989,471G/Tlikely benign
rs12539521365:33,989,473C/Tuncertain significance
rs7592173375:33,989,479T/Cuncertain significance
rs7669524505:33,989,482C/Tuncertain significance
rs7521875295:33,989,483G/Alikely benign
rs24789529515:33,989,491T/Cuncertain significance
rs21120329375:33,989,498C/Tuncertain significance

Showing 100 of 344 variants. Use the SNP search for the full list.

Gene information from NCBI Gene. Variant classifications from ClinVar.