ECH1

enoyl-CoA hydratase 1

Summary

This gene encodes a member of the hydratase/isomerase superfamily. The gene product shows high sequence similarity to enoyl-coenzyme A (CoA) hydratases of several species, particularly within a conserved domain characteristic of these proteins. The encoded protein, which contains a C-terminal peroxisomal targeting sequence, localizes to the peroxisome. The rat ortholog, which localizes to the matrix of both the peroxisome and mitochondria, can isomerize 3-trans,5-cis-dienoyl-CoA to 2-trans,4-trans-dienoyl-CoA, indicating that it is a delta3,5-delta2,4-dienoyl-CoA isomerase. This enzyme functions in the auxiliary step of the fatty acid beta-oxidation pathway. Expression of the rat gene is induced by peroxisome proliferators. [provided by RefSeq, Jul 2008]

Known Variants29 total

rsidPosition (GRCh37)AllelesClassClinVar
rs197155917119:39,306,263G/Auncertain significance
rs56727519619:39,306,353G/Auncertain significance
rs5875043119:39,306,529C/Tbenign
rs77859445019:39,306,600A/Guncertain significance
rs77749026019:39,306,604C/Tuncertain significance
rs251577109019:39,306,959C/Guncertain significance
rs74597213719:39,306,967G/Auncertain significance
rs222925919:39,307,103C/Tbenign
rs15006065019:39,308,054G/Aupstream gene variant
rs18610196119:39,308,096C/Tuncertain significance
rs76301831719:39,308,102C/Tuncertain significance
rs76602672819:39,308,132T/Auncertain significance
rs37437296719:39,308,145C/Guncertain significance
rs100265437419:39,308,173T/Cuncertain significance
rs1557019:39,308,190C/Tbenign
rs101183939819:39,308,215C/Tuncertain significance
rs7999460419:39,321,691G/Cbenign
rs20152695519:39,321,709G/Cuncertain significance
rs93740005619:39,321,723G/Auncertain significance
rs251334126719:39,321,764G/Cuncertain significance
rs74709361119:39,321,783T/Cuncertain significance
rs251334130719:39,321,784T/Guncertain significance
rs77548356619:39,321,803C/Auncertain significance
rs147761019019:39,322,003T/Cuncertain significance
rs941919:39,322,087G/Tbenign
rs251334195219:39,322,093T/Cuncertain significance
rs77055284919:39,322,121T/Auncertain significance
rs148130710319:39,322,132C/Tuncertain significance
rs11782073219:39,322,414T/Gbenign

Gene information from NCBI Gene. Variant classifications from ClinVar.