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

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

Gene information from NCBI Gene. Variant classifications from ClinVar.