F13A1

coagulation factor XIII A chain

Summary

This gene encodes the coagulation factor XIII A subunit. Coagulation factor XIII is the last zymogen to become activated in the blood coagulation cascade. Plasma factor XIII is a heterotetramer composed of 2 A subunits and 2 B subunits. The A subunits have catalytic function, and the B subunits do not have enzymatic activity and may serve as plasma carrier molecules. Platelet factor XIII is comprised only of 2 A subunits, which are identical to those of plasma origin. Upon cleavage of the activation peptide by thrombin and in the presence of calcium ion, the plasma factor XIII dissociates its B subunits and yields the same active enzyme, factor XIIIa, as platelet factor XIII. This enzyme acts as a transglutaminase to catalyze the formation of gamma-glutamyl-epsilon-lysine crosslinking between fibrin molecules, thus stabilizing the fibrin clot. It also crosslinks alpha-2-plasmin inhibitor, or fibronectin, to the alpha chains of fibrin. Factor XIII deficiency is classified into two categories: type I deficiency, characterized by the lack of both the A and B subunits; and type II deficiency, characterized by the lack of the A subunit alone. These defects can result in a lifelong bleeding tendency, defective wound healing, and habitual abortion. [provided by RefSeq, Jul 2008]

Known Variants216 total

rsidPosition (GRCh37)AllelesClassClinVar
rs17602344176:6,144,397C/Guncertain significance
rs18036046:6,144,599A/Guncertain significance
rs8860616556:6,144,685A/Cuncertain significance
rs9335697686:6,144,717C/Tuncertain significance
rs30244876:6,144,807G/Cuncertain significance
rs5483233826:6,144,822C/Auncertain significance
rs30244666:6,144,857C/Abenign
rs1386295636:6,144,886C/Auncertain significance
rs7747376616:6,144,907A/Guncertain significance
rs9219322006:6,144,918C/Tuncertain significance
rs7463641896:6,144,925C/Tuncertain significance
rs560390316:6,145,056C/Tuncertain significance
rs128626:6,145,109C/Tbenign
rs8860616566:6,145,224C/Tuncertain significance
rs8860616576:6,145,225C/Tuncertain significance
rs1915695856:6,145,233G/Auncertain significance
rs1489076106:6,145,246A/Guncertain significance
rs759816866:6,145,265C/Tuncertain significance
rs30244866:6,145,409C/Tlikely benign
rs1381399076:6,145,424G/Tuncertain significance
rs8860616586:6,145,435C/Auncertain significance
rs5687809786:6,145,582G/Auncertain significance
rs30244646:6,145,631C/Tbenign
rs1922348046:6,145,644A/Guncertain significance
rs10507836:6,145,692C/T3 prime UTR variantbenign
rs10507826:6,145,726A/Gbenign
rs30244636:6,145,740C/Alikely benign
rs5415964696:6,145,750C/Tuncertain significance
rs30244626:6,145,763T/Cbenign
rs1824255466:6,145,785G/Auncertain significance
rs8860616596:6,145,810C/Tuncertain significance
rs7712135346:6,145,856A/Tuncertain significance
rs59896:6,145,882G/Aconflicting classifications of pathogenicity
rs24804801396:6,145,901T/Cuncertain significance
rs2676067876:6,145,941G/Amissense variantpathogenic
rs13154904766:6,145,956A/Cuncertain significance
rs7625043956:6,145,983C/Tuncertain significance
rs7509715786:6,145,984G/Auncertain significance
rs17602663656:6,145,985G/Auncertain significance
rs30244606:6,146,321T/Cbenign
rs30244596:6,151,819C/Tbenign
rs30244586:6,151,952C/Tbenign
rs59806:6,152,007C/Tbenign
rs1219130646:6,152,046C/Tmissense variantpathogenic
rs2013022476:6,152,055T/Auncertain significance
rs3719641826:6,152,062T/Cuncertain significance
rs17603835956:6,152,093T/Cuncertain significance
rs2676067896:6,152,107G/Astop gainedpathogenic
rs7696568566:6,152,110G/Cuncertain significance
rs24804903106:6,152,131T/Auncertain significance
rs59886:6,152,137C/Gmissense variantbenign
rs59876:6,152,140C/Tmissense variantbenign
rs14837936826:6,152,182C/Tuncertain significance
rs22743946:6,152,211C/Gbenign
rs30244576:6,152,408C/Abenign
rs30244566:6,152,470A/Tbenign
rs4350486:6,167,621G/Abenign
rs2008301736:6,167,720C/Tuncertain significance
rs24805174346:6,167,734T/Cuncertain significance
rs1414168396:6,167,735T/Cuncertain significance
rs9582367166:6,167,736G/Alikely benign
rs1451803586:6,167,738C/Auncertain significance
rs1389435146:6,167,739C/Gconflicting classifications of pathogenicity
rs1437690716:6,167,766A/Glikely benign
rs7772802566:6,167,767G/Cuncertain significance
rs1482079956:6,167,791G/Auncertain significance
rs7571728386:6,167,795G/Alikely pathogenic
rs24805176326:6,167,803A/Guncertain significance
rs12773877416:6,167,817C/Guncertain significance
rs5299854696:6,167,819C/Tuncertain significance
rs1387544176:6,167,822C/Tconflicting classifications of pathogenicity
rs59836:6,167,833A/Tconflicting classifications of pathogenicity
rs3774793236:6,167,838C/Tuncertain significance
rs7463716326:6,167,858G/Cuncertain significance
rs69346086:6,169,890T/Gdownstream gene variant
rs30244466:6,174,581G/Tbenign
rs3800586:6,174,786G/Cbenign
rs24805294946:6,174,823C/Tlikely benign
rs1379451796:6,174,829C/Guncertain significance
rs1437115626:6,174,830G/Aconflicting classifications of pathogenicity
rs617344866:6,174,834C/Tuncertain significance
rs5592162226:6,174,841C/Tlikely benign
rs1135999406:6,174,842G/Alikely benign
rs59866:6,174,856C/Tlikely benign
rs3726514616:6,174,865C/Tlikely benign
rs59826:6,174,866G/Aconflicting classifications of pathogenicity
rs583157456:6,174,871C/Tlikely benign
rs1219130696:6,174,873C/Tmissense variantpathogenic
rs7484133076:6,174,884G/Tuncertain significance
rs59846:6,174,908G/Alikely benign
rs3676793576:6,174,938C/Tuncertain significance
rs1428917016:6,174,940G/Alikely benign
rs13804479346:6,175,022A/Tuncertain significance
rs11784154196:6,175,029C/Tuncertain significance
rs1219130686:6,175,056C/Tmissense variantpathogenic
rs3720363576:6,175,057G/Tpathogenic
rs7458883616:6,175,068C/Auncertain significance
rs7647535616:6,175,098G/Cuncertain significance
rs30244446:6,175,174C/Tbenign
rs37783556:6,177,028G/T

Showing 100 of 216 variants. Use the SNP search for the full list.

Gene information from NCBI Gene. Variant classifications from ClinVar.