PHYKPL

5-phosphohydroxy-L-lysine phospho-lyase

Summary

This is a nuclear gene encoding a mitochondrial enzyme that catalyzes the conversion of 5-phosphonooxy-L-lysine to ammonia, inorganic phosphate, and 2-aminoadipate semialdehyde. Mutations in this gene may cause phosphohydroxylysinuria. Alternative splicing results in multiple transcript variants. [provided by RefSeq, May 2013]

Known Variants54 total

rsidPosition (GRCh37)AllelesClassClinVar
rs17585768595:177,638,945C/Glikely benign
rs3740484795:177,638,960T/Cuncertain significance
rs1421815175:177,638,965T/Amissense variantuncertain significance
rs14591688615:177,640,009C/Tlikely benign
rs1415233945:177,640,034T/Cmissense variant
rs1507221245:177,640,044A/Tuncertain significance
rs3775964255:177,640,076T/Cuncertain significance
rs7641156965:177,640,088C/Tlikely benign
rs1499608505:177,640,101C/Tlikely benign
rs5743854625:177,641,194G/A
rs7626580375:177,641,813C/Auncertain significance
rs11588442955:177,641,855T/Auncertain significance
rs1423857615:177,641,870T/Cuncertain significance
rs1486582185:177,642,291G/Alikely benign
rs2004964495:177,642,317C/Tlikely benign
rs14221576265:177,642,323G/Auncertain significance
rs3753193275:177,642,392C/Tuncertain significance
rs7676430055:177,642,412G/Auncertain significance
rs412855795:177,649,103A/G
rs3679087335:177,649,376C/Tuncertain significance
rs14043012475:177,649,412C/Tlikely benign
rs7756634435:177,649,486C/Tuncertain significance
rs3736754665:177,649,523G/Cuncertain significance
rs2011058575:177,649,565C/Tmissense variantpathogenic
rs7656168465:177,649,573C/Auncertain significance
rs1501084555:177,649,697G/Aintron variant
rs24812717075:177,649,871T/Cuncertain significance
rs14379532015:177,649,896C/Guncertain significance
rs1421424845:177,649,920C/Tbenign
rs7476441015:177,651,471C/Tuncertain significance
rs7705152635:177,651,483C/Tuncertain significance
rs24813473625:177,651,517T/Cuncertain significance
rs7812486825:177,651,531C/Tuncertain significance
rs1444229845:177,651,549G/Auncertain significance
rs7656754435:177,651,564G/Auncertain significance
rs3771157315:177,651,571G/Alikely benign
rs7579767125:177,651,678G/Auncertain significance
rs7783101215:177,651,683T/Auncertain significance
rs12779465175:177,651,705G/Cuncertain significance
rs1142985005:177,651,727C/Tlikely benign
rs7615969885:177,651,728G/Auncertain significance
rs7795420595:177,652,357G/Auncertain significance
rs77071475:177,652,392T/Cbenign
rs5376402635:177,652,395C/Tuncertain significance
rs7736722225:177,652,396G/Auncertain significance
rs17630561595:177,656,997C/Guncertain significance
rs7582909415:177,657,031C/Tuncertain significance
rs24815461985:177,657,086G/Auncertain significance
rs7703440675:177,658,477C/Tuncertain significance
rs37498065:177,659,429C/Aregulatory region variant
rs7718371535:177,659,494T/Cuncertain significance
rs7646788395:177,659,503G/Auncertain significance
rs1167357715:177,659,519C/Gbenign
rs13553871135:177,659,529T/Cuncertain significance

Gene information from NCBI Gene. Variant classifications from ClinVar.