Function | Essential for mitochondrial network organization, mitochondrial metabolism and cell growth at organism and cellular level. May play an important role in mitochondrial protein synthesis. May also participate in mitochondrial DNA replication. May bind to mitochondrial DNA D-loops and contribute to nucleoid stability. Required for enhanced channeling of cholesterol for hormone-dependent steroidogenesis. Involved in mitochondrial-mediated antiviral innate immunity. Required to protect mitochondria from the PERK-mediated unfolded protein response: specifically inhibits the activity of EIF2AK3/PERK at mitochondria-endoplasmic reticulum contact sites, thereby providing a safe haven for mitochondrial protein translation during endoplasmic reticulum stress. Ability to inhibit EIF2AK3/PERK is independent of its ATPase activity. Also involved in the mitochondrial DNA damage response by promoting signaling between damaged genomes and the mitochondrial membrane, leading to activation of the integrated stress response (ISR). |
Protein Name | Atpase Family Aaa Domain-Containing Protein 3a |
Cellular Localisation | Mitochondrion Inner MembraneSingle-Pass Membrane ProteinMitochondrion MatrixMitochondrion NucleoidIn The Mitochondrial Inner MembraneEnriched In Sites With The Potential To Form Contacts With The Outer MembraneThe N-Terminal Domain Interacts With The Inner Surface Of The Mitochondrial Outer Membrane And The C-Terminal Domain Localizes In A Specific Matrix CompartmentWhere It Is Associated With NucleoidsAlso Present At Mitochondria-Endoplasmic Reticulum Contact SitesWhere It Interacts With Eif2ak3/Perk |
Alternative Antibody Names | Anti-Atpase Family Aaa Domain-Containing Protein 3a antibodyAnti-ATAD3A antibody |
Information sourced from Uniprot.org