PET100

PET100 cytochrome c oxidase chaperone

Summary

Mitochondrial complex IV, or cytochrome c oxidase, is a large transmembrane protein complex that is part of the respiratory electron transport chain of mitochondria. The small protein encoded by this gene plays a role in the biogenesis of mitochondrial complex IV. This protein localizes to the inner mitochondrial membrane and is exposed to the intermembrane space. Mutations in this gene are associated with mitochondrial complex IV deficiency. This gene has a pseudogene on chromosome 3. Alternative splicing results in multiple transcript variants. [provided by RefSeq, Apr 2014]

Known Variants78 total

rsidPosition (GRCh37)AllelesClassClinVar
rs413481019:7,694,349C/T—likely benign
rs413480919:7,694,393G/A—benign
rs413480819:7,694,538A/G—benign
rs7392147719:7,694,585G/T—benign
rs79408519:7,694,602C/G—benign
rs376067519:7,694,662T/C—benign
rs105752268219:7,694,701G/C—likely benign
rs100160385219:7,694,717G/A—likely benign
rs97739251219:7,694,720A/T—pathogenic
rs203123336419:7,694,737G/A—likely benign
rs251266194519:7,694,756C/T—likely benign
rs138404082619:7,694,757A/G—likely benign
rs203123458919:7,694,760G/A—likely benign
rs98970824819:7,694,761G/A—likely benign
rs11535707919:7,695,266G/C—benign
rs376067619:7,695,336A/G—benign
rs413480519:7,695,441C/T—likely benign
rs251266335319:7,695,445C/G—likely benign
rs203125769319:7,695,457A/G—likely pathogenic
rs131119310519:7,695,470C/A—pathogenic
rs203125886519:7,695,497G/A—pathogenic
rs18432585419:7,695,505A/G—likely benign
rs413480419:7,695,513G/C—uncertain significance
rs117200439319:7,695,546G/A—likely pathogenic
rs96339718419:7,695,549G/A—uncertain significance
rs75355491519:7,695,552A/G—conflicting classifications of pathogenicity
rs251266361619:7,695,559G/A—likely benign
rs214619308819:7,695,561G/C—likely benign
rs251266362919:7,695,563G/A—likely benign
rs1126000419:7,695,620T/C—benign
rs11762260219:7,695,636T/A—likely benign
rs14829638719:7,695,681G/A—likely benign
rs105158862419:7,695,700T/C—likely benign
rs145943789019:7,695,706C/G—conflicting classifications of pathogenicity
rs251266409419:7,695,708G/A—likely pathogenic
rs203126645419:7,695,733G/T—uncertain significance
rs75632614719:7,695,735A/T—uncertain significance
rs203126695319:7,695,743T/C—likely benign
rs251266418019:7,695,747C/T—likely benign
rs11563573019:7,695,758A/G—likely benign
rs1188182019:7,695,841A/G—benign
rs376067719:7,695,977C/G—benign
rs1188219719:7,696,138G/A—benign
rs11312465419:7,696,198G/A—likely benign
rs7392147919:7,696,260G/A—likely benign
rs54761646719:7,696,341C/T—likely benign
rs139280900319:7,696,342C/T—likely benign
rs141117191919:7,696,343G/T—likely benign
rs105752229419:7,696,344C/T—likely benign
rs251266581419:7,696,349C/T—likely benign
rs251266582019:7,696,350C/T—likely benign
rs135566594519:7,696,352C/T—likely benign
rs251266585019:7,696,355C/G—likely benign
rs251266586519:7,696,359C/T—uncertain significance
rs130334259319:7,696,361T/C—likely benign
rs100350500619:7,696,380A/C—uncertain significance
rs75620125719:7,696,392C/T—uncertain significance
rs76428872219:7,696,393G/A—uncertain significance
rs75407118719:7,696,394G/A—likely benign
rs75738827519:7,696,398C/T—uncertain significance
rs77924353319:7,696,399G/A—conflicting classifications of pathogenicity
rs96864402119:7,696,401C/T—uncertain significance
rs97721992619:7,696,404G/T—uncertain significance
rs74620453119:7,696,405A/T—uncertain significance
rs122216405719:7,696,406G/A—likely benign
rs118664966019:7,696,413C/T—uncertain significance
rs57062017719:7,696,417T/C—conflicting classifications of pathogenicity
rs123304964719:7,696,418T/C—likely benign
rs11766171519:7,696,419C/T—benign
rs55638045119:7,696,420G/A—conflicting classifications of pathogenicity
rs37596893519:7,696,421C/T—likely benign
rs76810998619:7,696,424C/T—likely benign
rs203130267519:7,696,427C/T—likely benign
rs251266609819:7,696,436C/T—likely benign
rs127091155719:7,696,438C/G—likely benign
rs136136001819:7,696,441G/A—likely benign
rs11460085819:7,696,496G/T—likely benign
rs11236778119:7,696,678C/T—likely benign

Gene information from NCBI Gene. Variant classifications from ClinVar.