PSMC3

proteasome 26S subunit, ATPase 3

Summary

The 26S proteasome is a multicatalytic proteinase complex with a highly ordered structure composed of 2 complexes, a 20S core and a 19S regulator. The 20S core is composed of 4 rings of 28 non-identical subunits; 2 rings are composed of 7 alpha subunits and 2 rings are composed of 7 beta subunits. The 19S regulator is composed of a base, which contains 6 ATPase subunits and 2 non-ATPase subunits, and a lid, which contains up to 10 non-ATPase subunits. Proteasomes are distributed throughout eukaryotic cells at a high concentration and cleave peptides in an ATP/ubiquitin-dependent process in a non-lysosomal pathway. An essential function of a modified proteasome, the immunoproteasome, is the processing of class I MHC peptides. This gene encodes one of the ATPase subunits, a member of the triple-A family of ATPases that have chaperone-like activity. This subunit may compete with PSMC2 for binding to the HIV tat protein to regulate the interaction between the viral protein and the transcription complex. A pseudogene has been identified on chromosome 9. [provided by RefSeq, Jul 2008]

Known Variants24 total

rsidPosition (GRCh37)AllelesClassClinVar
rs76054688611:47,440,455G/Alikely benign
rs249604714811:47,440,672C/Guncertain significance
rs229357911:47,440,758G/Adownstream gene variant
rs712041311:47,441,335A/Gdownstream gene variant
rs117789807111:47,441,478T/Cpathogenic
rs1083870811:47,441,513G/Adownstream gene variant
rs224251111:47,441,664C/Tregulatory region variant
rs52727634611:47,441,877C/Tlikely benign
rs249605018711:47,441,902C/Tuncertain significance
rs15108194211:47,441,928C/Tlikely benign
rs249605099111:47,442,234A/Glikely pathogenic
rs136334850011:47,442,253G/Apathogenic
rs14265188511:47,444,155A/Guncertain significance
rs249605563511:47,444,183T/Cuncertain significance
rs249605570711:47,444,227A/Gconflicting classifications of pathogenicity
rs134775354311:47,444,254G/Auncertain significance
rs14805495811:47,444,393G/Alikely benign
rs249605605411:47,444,404G/Aconflicting classifications of pathogenicity
rs249605827011:47,445,631C/Tuncertain significance
rs134487157611:47,446,217T/Cuncertain significance
rs76885845411:47,446,681G/Alikely benign
rs1779080411:47,447,202T/Cintron variant
rs104035847911:47,447,813A/Glikely benign
rs794870511:47,447,955C/Gregulatory region variant

Gene information from NCBI Gene. Variant classifications from ClinVar.