UROC1

urocanate hydratase 1

Summary

This gene encodes an enzyme involved in the second step of histidine catabolism, metabolizing urocanic acid to formiminoglutamic acid. Deficiency of this enzyme results in urocanic aciduria, and is an apparent cause of mental retardation. Multiple transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Apr 2021]

Known Variants126 total

rsidPosition (GRCh37)AllelesClassClinVar
rs1997127313:126,201,221G/Tuncertain significance
rs1458910233:126,201,278G/Alikely benign
rs1490081303:126,201,291T/Clikely benign
rs5761575813:126,201,319G/Auncertain significance
rs3749586553:126,201,321C/Tuncertain significance
rs3686288783:126,202,216T/Cuncertain significance
rs7474520503:126,202,256C/Tuncertain significance
rs16874773:126,202,257A/Gbenign
rs5405568593:126,202,264G/Auncertain significance
rs5459142983:126,202,272G/Alikely benign
rs7501424453:126,202,287G/Alikely benign
rs7726744703:126,202,290T/Clikely benign
rs1450691293:126,202,295C/Tuncertain significance
rs8968813013:126,202,319A/Clikely benign
rs1507704243:126,207,048C/Tuncertain significance
rs1391206433:126,207,049G/Alikely benign
rs1495412323:126,207,058G/Alikely benign
rs5575387723:126,207,085G/Alikely benign
rs7739169993:126,207,102C/Tuncertain significance
rs7549603943:126,207,126C/Tlikely benign
rs3767573233:126,207,127G/Auncertain significance
rs12080909363:126,208,124C/Tuncertain significance
rs1441148753:126,208,137C/Tuncertain significance
rs13639663683:126,208,151G/Tuncertain significance
rs1503261223:126,208,174G/Abenign
rs3681084223:126,208,181C/Tuncertain significance
rs1996374823:126,208,186G/Alikely benign
rs3768410693:126,208,214G/Auncertain significance
rs7774793:126,208,249C/Abenign
rs355165123:126,211,278C/Tconflicting classifications of pathogenicity
rs7774743:126,211,282G/Abenign
rs2012696143:126,211,314G/Auncertain significance
rs1162864323:126,211,365G/Abenign
rs566817253:126,211,367G/Abenign
rs7738782713:126,211,369G/Alikely benign
rs7486807903:126,214,895C/Tuncertain significance
rs7713076823:126,214,899C/Auncertain significance
rs756039883:126,214,903C/Tbenign
rs7629271153:126,214,912C/Tuncertain significance
rs3775494143:126,214,918T/Cuncertain significance
rs7598825833:126,214,922C/Tuncertain significance
rs7508477393:126,214,940C/Tuncertain significance
rs24733553293:126,214,945A/Glikely benign
rs11959665593:126,214,947C/Tlikely benign
rs7506525073:126,216,905A/Guncertain significance
rs1156265013:126,216,925T/Cbenign
rs7788855253:126,216,929G/Auncertain significance
rs1489553973:126,216,967C/Tlikely benign
rs1378527953:126,216,984G/Amissense variantpathogenic
rs1480404973:126,218,196C/Tuncertain significance
rs7654566333:126,218,198T/Cuncertain significance
rs14070971363:126,218,205G/Cuncertain significance
rs2017249973:126,218,210C/Tuncertain significance
rs98716713:126,218,211G/Abenign
rs7576791093:126,218,237T/Cuncertain significance
rs1389213393:126,218,249G/Auncertain significance
rs1473535703:126,218,934G/Alikely benign
rs24733635983:126,218,937G/Cuncertain significance
rs5474248303:126,218,960C/Tlikely benign
rs130724043:126,219,030T/Cbenign
rs7543464913:126,219,597C/Tlikely benign
rs1499086493:126,219,598G/Auncertain significance
rs24733648213:126,219,616G/Auncertain significance
rs340259263:126,219,627G/Abenign
rs7670338143:126,219,660T/Alikely benign
rs7658477643:126,219,687C/Tlikely benign
rs7456785353:126,219,702G/Alikely benign
rs3773094713:126,219,713G/Aconflicting classifications of pathogenicity
rs19359153283:126,220,118G/Tuncertain significance
rs7525668713:126,220,152C/Tlikely benign
rs3720662843:126,220,751C/Tlikely benign
rs3722907503:126,222,857G/Auncertain significance
rs7816219253:126,222,885C/Tpathogenic
rs2022326113:126,222,886C/Tconflicting classifications of pathogenicity
rs3678799243:126,222,901C/Auncertain significance
rs3733227663:126,222,902G/Auncertain significance
rs2002331353:126,224,550T/Cuncertain significance
rs5552157963:126,224,571G/Alikely benign
rs5374076993:126,224,585T/Cuncertain significance
rs7454507043:126,224,586G/Alikely benign
rs1404326073:126,224,606C/Tuncertain significance
rs7608790463:126,224,609C/Tuncertain significance
rs13065121213:126,224,620G/Auncertain significance
rs10052227523:126,224,630C/Auncertain significance
rs1504371493:126,224,648C/Gconflicting classifications of pathogenicity
rs7812496853:126,224,665C/Tuncertain significance
rs7621894703:126,224,681C/Tuncertain significance
rs7704304933:126,224,790C/Guncertain significance
rs3775945423:126,224,802C/Tuncertain significance
rs7516175013:126,224,817C/Tuncertain significance
rs7676683653:126,224,820T/Auncertain significance
rs8995676693:126,224,842C/Guncertain significance
rs1382067163:126,224,849C/Tlikely benign
rs7783201853:126,224,850C/Tuncertain significance
rs1490969633:126,224,851G/Alikely benign
rs10108380443:126,226,613C/Tuncertain significance
rs1459480283:126,226,621G/Alikely benign
rs1437820373:126,226,624C/Tlikely benign
rs344880363:126,226,625G/Alikely benign
rs7781887683:126,226,843G/Alikely benign

Showing 100 of 126 variants. Use the SNP search for the full list.

Gene information from NCBI Gene. Variant classifications from ClinVar.