Protein: Ubiquitin-like-conjugating enzyme ATG3 (EC 2.3.2.-) (Autophagy-related protein 3) (APG3-like) (hApg3) (Protein PC3-96) (Q9NT62)
Organism: Homo sapiens (Human)
Function: E2 conjugating enzyme required for the cytoplasm to vacuole transport (Cvt), autophagy, and mitochondrial homeostasis. Responsible for the E2-like covalent binding of phosphatidylethanolamine to the C-terminal Gly of ATG8-like proteins (GABARAP, GABARAPL1, GABARAPL2 or MAP1LC3A). The ATG12-ATG5 conjugate plays a role of an E3 and promotes the transfer of ATG8-like proteins from ATG3 to phosphatidylethanolamine (PE). This step is required for the membrane association of ATG8-like proteins. The formation of the ATG8-phosphatidylethanolamine conjugates is essential for autophagy and for the cytoplasm to vacuole transport (Cvt). Preferred substrate is MAP1LC3A. Also acts as an autocatalytic E2-like enzyme, catalyzing the conjugation of ATG12 to itself, ATG12 conjugation to ATG3 playing a role in mitochondrial homeostasis but not in autophagy. ATG7 (E1-like enzyme) facilitates this reaction by forming an E1-E2 complex with ATG3. Promotes primary ciliogenesis by removing OFD1 from centriolar satellites via the autophagic pathway.
Subcellular location: Cytoplasm {ECO:0000269|PubMed:11825910}.
Sequence: MQNVINTVKGKALEVAEYLTPVLKESKFKETGVITPEEFVAAGDHLVHHCPTWQWATGEELKVKAYLPTGKQFLVTKNVPCYKRCKQMEYSDELEAIIEEDDGDGGWVDTYHNTGITGITEAVKEITLENKDNIRLQDCSALCEEEEDEDEGEAADMEEYEESGLLETDEATLDTRKIVEACKAKTDAGGEDAILQTRTYDLYITYDKYYQTPRLWLFGYDEQRQPLTVEHMYEDISQDHVKKTVTIENHPHLPPPPMCSVHPCRHAEVMKKIIETVAEGGGELGVHMYLLIFLKFVQAVIPTIEYDYTRHFTM
InterPro domains: - IPR007135: Ubiquitin-like-conjugating enzyme Atg3/Atg10 (family) [38-308]
Interaction partners: - Gamma-aminobutyric acid receptor-associated protein-like 2
- Autophagy protein 5
- Ubiquitin-like protein ATG12
- Autophagy-related protein 16-1
- Ubiquitin-like modifier-activating enzyme ATG7
- Gamma-aminobutyric acid receptor-associated protein
- GABA type A receptor associated protein like 1
- Microtubule-associated protein 1 light chain 3 beta
- Microtubule-associated protein 1 light chain 3 alpha
- CASP8 and FADD-like apoptosis regulator
Gene Ontology annotations:
Molecular Function (MF): GO:0003674 (molecular function), GO:0005488 (binding), GO:0005515 (protein binding)
Biological Process (BP): GO:0008150 (biological process), GO:0008152 (metabolic process), GO:0065007 (biological regulation), GO:0050789 (regulation of biological process), GO:0009987 (cellular process), GO:0048519 (negative regulation of biological process), GO:0044237 (cellular metabolic process), GO:0051051 (negative regulation of transport), GO:0008219 (cell death), GO:0032879 (regulation of localization), GO:0050794 (regulation of cellular process), GO:0071840 (cellular component organization or biogenesis), GO:0061919 (process utilizing autophagic mechanism), GO:0009056 (catabolic process), GO:0012501 (programmed cell death), GO:0051049 (regulation of transport), GO:0044085 (cellular component biogenesis), GO:0044248 (cellular catabolic process), GO:0016043 (cellular component organization), GO:0060627 (regulation of vesicle-mediated transport), GO:0051128 (regulation of cellular component organization), GO:0050765 (negative regulation of phagocytosis), GO:0006914 (autophagy), GO:0044087 (regulation of cellular component biogenesis), GO:0016236 (macroautophagy), GO:0050764 (regulation of phagocytosis), GO:0031344 (regulation of cell projection organization), GO:0006915 (apoptotic process), GO:0033043 (regulation of organelle organization), GO:0006996 (organelle organization), GO:1902115 (regulation of organelle assembly), GO:0060491 (regulation of cell projection assembly), GO:0022607 (cellular component assembly), GO:0070925 (organelle assembly), GO:0007005 (mitochondrion organization), GO:0120032 (regulation of plasma membrane bounded cell projection assembly), GO:0007033 (vacuole organization), GO:0120035 (regulation of plasma membrane bounded cell projection organization), GO:1905037 (autophagosome organization), GO:1902017 (regulation of cilium assembly), GO:0008637 (apoptotic mitochondrial changes), GO:0000045 (autophagosome assembly)
Cellular Component (CC): GO:0005575 (cellular component), GO:0110165 (cellular anatomical entity), GO:0005622 (intracellular anatomical structure), GO:0043226 (organelle), GO:0005829 (cytosol), GO:0005737 (cytoplasm), GO:0031974 (membrane-enclosed lumen), GO:0005654 (nucleoplasm), GO:0043233 (organelle lumen), GO:0043229 (intracellular organelle), GO:0043227 (membrane-bounded organelle), GO:0043231 (intracellular membrane-bounded organelle), GO:0070013 (intracellular organelle lumen), GO:0031981 (nuclear lumen), GO:0005634 (nucleus)