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MT001259

UniProt Annotations

Entry Information

Gene Name	autophagy related 3
Protein Entry	ATG3_HUMAN
UniProt ID	Q9NT62
Species	Human

Comments

Comment type	Description
Alternative Products	Event=Alternative splicing; Named isoforms=2; Name=1; IsoId=Q9NT62-1; Sequence=Displayed; Name=2; IsoId=Q9NT62-2; Sequence=VSP_013037; Note=No experimental confirmation available. Ref.4 (AAH02830) sequence differs from that shown due to a frameshift in position 311. {ECO:0000305};
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. {ECO:0000269\|PubMed:11825910, ECO:0000269\|PubMed:12207896, ECO:0000269\|PubMed:12890687, ECO:0000269\|PubMed:16704426, ECO:0000269\|PubMed:20723759}.
Interaction	O95166:GABARAP; NbExp=5; IntAct=EBI-988094, EBI-712001; Q9H0R8:GABARAPL1; NbExp=3; IntAct=EBI-988094, EBI-746969; P60520:GABARAPL2; NbExp=5; IntAct=EBI-988094, EBI-720116; Q9GZQ8:MAP1LC3B; NbExp=7; IntAct=EBI-988094, EBI-373144; Q7Z6L1:TECPR1; NbExp=3; IntAct=EBI-988094, EBI-2946676;
Ptm	Cleaved by CASP8 upon death ligand binding such as tumor necrosis factor-alpha. CASP8 cleavage blocks survival-related autophagy and favors apoptosis. {ECO:0000269\|PubMed:22644571}.
Ptm	Conjugated to ATG12 at Lys-243. ATG12-conjugation plays a role in regulation of mitochondrial homeostasis and cell death, while it is not involved in PE-conjugation to ATG8-like proteins and autophagy.
Sequence Caution	Sequence=AAH02830.1; Type=Frameshift; Positions=290; Evidence={ECO:0000305}; Sequence=BAB15237.1; Type=Erroneous initiation; Note=Translation N-terminally extended.; Evidence={ECO:0000305};
Similarity	Belongs to the ATG3 family. {ECO:0000305}.
Subcellular Location	Cytoplasm {ECO:0000269\|PubMed:11825910}.
Subunit	Interacts with ATG7 and ATG12. The complex, composed of ATG3 and ATG7, plays a role in the conjugation of ATG12 to ATG5. Interacts with FNBP1L. {ECO:0000269\|PubMed:11825910, ECO:0000269\|PubMed:12207896, ECO:0000269\|PubMed:19342671, ECO:0000269\|PubMed:20723759, ECO:0000269\|PubMed:22170151}.
Tissue Specificity	Widely expressed, with a highest expression in heart, skeletal muscle, kidney, liver and placenta. {ECO:0000269\|PubMed:11825910}.