HAAO

3-hydroxyanthranilate 3,4-dioxygenase

Summary

3-Hydroxyanthranilate 3,4-dioxygenase is a monomeric cytosolic protein belonging to the family of intramolecular dioxygenases containing nonheme ferrous iron. It is widely distributed in peripheral organs, such as liver and kidney, and is also present in low amounts in the central nervous system. HAAO catalyzes the synthesis of quinolinic acid (QUIN) from 3-hydroxyanthranilic acid. QUIN is an excitotoxin whose toxicity is mediated by its ability to activate glutamate N-methyl-D-aspartate receptors. Increased cerebral levels of QUIN may participate in the pathogenesis of neurologic and inflammatory disorders. HAAO has been suggested to play a role in disorders associated with altered tissue levels of QUIN. [provided by RefSeq, Jul 2008]

Known Variants41 total

rsidPosition (GRCh37)AllelesClassClinVar
rs49536572:42,993,782T/A
rs2005529802:42,994,581C/Auncertain significance
rs1462422292:42,994,647C/Guncertain significance
rs16707630722:42,994,771G/Auncertain significance
rs7688056152:42,994,777C/Guncertain significance
rs340531332:42,994,814C/Guncertain significance
rs1505374162:42,995,018G/Auncertain significance
rs3728524372:42,995,041C/Tuncertain significance
rs1130198652:42,995,058T/Cbenign
rs37555402:42,995,840T/G
rs134135072:42,996,819A/Gbenign
rs1453374032:42,996,907C/Tlikely benign
rs24668600982:42,996,917C/Tuncertain significance
rs11354017432:42,996,925C/Tpathogenic
rs1423005892:42,996,959C/Guncertain significance
rs22418502:42,997,614G/Abenign
rs24668632002:42,997,642A/Guncertain significance
rs7535039702:42,997,661C/Tuncertain significance
rs14433360142:42,997,667C/Tuncertain significance
rs12918691782:42,997,690A/Guncertain significance
rs13639545562:43,010,481C/Tlikely pathogenic
rs16721523822:43,010,503C/Alikely pathogenic
rs7591037772:43,010,506C/Tlikely benign
rs12442248152:43,010,515C/Tuncertain significance
rs24668966922:43,010,553A/Glikely pathogenic
rs12320962912:43,010,923C/Tlikely pathogenic
rs7800637632:43,010,934C/Tuncertain significance
rs3690055642:43,010,941C/Guncertain significance
rs341832202:43,010,950G/Auncertain significance
rs1996526252:43,010,968G/Cuncertain significance
rs1495165352:43,010,973C/Tlikely benign
rs14185705262:43,010,981C/Tuncertain significance
rs1409418262:43,015,684G/Abenign
rs7467028522:43,015,687G/Tpathogenic
rs16725588412:43,015,700C/Tconflicting classifications of pathogenicity
rs10487684112:43,015,703G/Clikely benign
rs38161822:43,015,704T/Abenign
rs14541755012:43,015,710G/Auncertain significance
rs1148943332:43,015,717G/Abenign
rs38161832:43,015,719T/Cmissense variantbenign
rs38161842:43,015,757G/Abenign

Gene information from NCBI Gene. Variant classifications from ClinVar.