ECE1

endothelin converting enzyme 1

Summary

The protein encoded by this gene is involved in proteolytic processing of endothelin precursors to biologically active peptides. Mutations in this gene are associated with Hirschsprung disease, cardiac defects and autonomic dysfunction. Alternatively spliced transcript variants encoding different isoforms have been noted for this gene.[provided by RefSeq, Sep 2009]

Known Variants81 total

rsidPosition (GRCh37)AllelesClassClinVar
rs25511882431:21,544,228G/Alikely benign
rs743432471:21,544,247T/Abenign
rs30269061:21,546,501G/Amissense variantpathogenic
rs7719423761:21,546,574G/Alikely benign
rs25511914251:21,546,597T/Cuncertain significance
rs7572079561:21,546,608C/Tuncertain significance
rs25511966441:21,551,764C/Alikely benign
rs3678124361:21,551,817C/Tlikely pathogenic
rs1408329031:21,551,823C/Tlikely benign
rs10295386581:21,551,872G/Clikely benign
rs1997335571:21,551,899T/Glikely benign
rs2008947511:21,551,904G/Alikely pathogenic
rs7592294791:21,551,917C/Tlikely benign
rs25511969271:21,551,919T/Guncertain significance
rs7601716131:21,551,930C/Tuncertain significance
rs30269031:21,553,723G/Tbenign
rs11703016531:21,554,452C/Guncertain significance
rs7529793421:21,554,464C/Tuncertain significance
rs1490214291:21,554,495C/Tbenign
rs10151017881:21,554,519C/Tlikely benign
rs20981843961:21,560,052G/Cuncertain significance
rs22283211:21,560,080A/Gbenign
rs9723546661:21,560,120G/Auncertain significance
rs283680181:21,562,343G/Alikely benign
rs25512113791:21,562,344T/Cuncertain significance
rs1438198961:21,562,363G/Alikely benign
rs1447372091:21,563,282A/Guncertain significance
rs1411468851:21,564,631C/Tlikely benign
rs1466551541:21,564,671G/Cuncertain significance
rs1484616601:21,564,860C/Tregulatory region variant
rs283680041:21,571,475A/Gbenign
rs3721138941:21,571,562G/Auncertain significance
rs1453013951:21,571,570T/Cbenign
rs125621971:21,571,601G/Abenign
rs413191521:21,573,705T/Glikely benign
rs20982049651:21,573,720T/Cuncertain significance
rs22294501:21,573,722G/Abenign
rs20982050461:21,573,765T/Cuncertain significance
rs7743320091:21,573,784C/Tuncertain significance
rs21032638071:21,573,805G/Tuncertain significance
rs7657637041:21,573,810A/Clikely pathogenic
rs1393264671:21,573,817T/Cbenign
rs10766691:21,573,855G/Abenign
rs2125171:21,577,159T/C
rs2125221:21,582,425C/Tbenign
rs8675479491:21,582,525G/Auncertain significance
rs1453115941:21,582,583C/Tuncertain significance
rs1468757841:21,582,599C/Auncertain significance
rs9157250191:21,584,009C/Tlikely benign
rs283679841:21,584,010A/Gbenign
rs7639385231:21,584,039C/Tlikely benign
rs12021155071:21,584,050C/Auncertain significance
rs7633054861:21,585,219G/Alikely benign
rs5516290691:21,585,265G/Auncertain significance
rs1429884241:21,586,808T/Cuncertain significance
rs1506440741:21,586,831C/Tuncertain significance
rs1474758631:21,586,862C/Tlikely benign
rs133063131:21,586,875C/Tbenign
rs14818513621:21,599,222G/Tuncertain significance
rs2015181591:21,599,301G/Alikely benign
rs15533647181:21,605,747C/Tuncertain significance
rs1995212001:21,605,750A/Cconflicting classifications of pathogenicity
rs1996662821:21,605,779C/Tuncertain significance
rs5506403151:21,605,807G/Cuncertain significance
rs5369752101:21,605,860G/Alikely benign
rs5573651611:21,616,556C/Tlikely benign
rs12890687921:21,616,570C/Tuncertain significance
rs1425327541:21,616,571C/Guncertain significance
rs13513738101:21,616,597A/Guncertain significance
rs25447709981:21,616,631C/Tuncertain significance
rs13989787581:21,616,864G/Auncertain significance
rs10241434461:21,616,865C/Tuncertain significance
rs1930173311:21,616,884C/Tbenign
rs20982654441:21,616,889G/Auncertain significance
rs2130451:21,617,245G/Tregulatory region variantrisk factor
rs2130461:21,617,746A/Cupstream gene variantrisk factor
rs127378591:21,630,291C/Tdownstream gene variant
rs30268271:21,651,179G/Aregulatory region variant
rs2130321:21,654,735T/Cintron variant
rs1149215871:21,671,973G/Aregulatory region variant
rs1462536131:21,672,873T/Gregulatory region variant

Gene information from NCBI Gene. Variant classifications from ClinVar.