MitoProteome Database

MT000749

UniProt Annotations

Entry Information
Gene Namecollagen, type IV, alpha 3 (Goodpasture antigen) binding protein
Protein EntryC43BP_HUMAN
UniProt IDQ9Y5P4
SpeciesHuman
Comments
Comment typeDescription
Alternative Products Event=Alternative splicing; Named isoforms=3; Name=1; Synonyms=CERTL; IsoId=Q9Y5P4-1; Sequence=Displayed; Name=2; Synonyms=Delta26, GPBPD26; IsoId=Q9Y5P4-2; Sequence=VSP_006276; Name=3; IsoId=Q9Y5P4-3; Sequence=VSP_041022;
Caution Was originally reported to have a protein kinase activity and to phosphorylate on Ser and Thr residues the Goodpasture autoantigen (in vitro). {ECO:0000305|PubMed:10212244}.
Domain The FFAT motif is required for interaction with VAPA and VAPB. {ECO:0000269|PubMed:14685229}.
Domain The PH domain targets the Golgi apparatus. {ECO:0000269|PubMed:14685229}.
Domain The START domain recognizes ceramides and diacylglycerol lipids, interacts with membranes, and mediates the intermembrane transfer of ceramides and diacylglycerol lipids. {ECO:0000269|PubMed:14685229}.
Function Shelters ceramides and diacylglycerol lipids inside its START domain and mediates the intracellular trafficking of ceramides and diacylglycerol lipids in a non-vesicular manner. {ECO:0000269|PubMed:14685229, ECO:0000269|PubMed:17591919, ECO:0000269|PubMed:18184806, ECO:0000269|PubMed:20036255}.
Ptm Phosphorylation on Ser-132 decreases the affinity toward phosphatidylinositol 4-phosphate at Golgi membranes and reduces ceramide transfer activity. Inactivated by hyperphosphorylation of serine residues by CSNK1G2/CK1 that triggers dissociation from the Golgi complex, thus down-regulating ER-to-Golgi transport of ceramide and sphingomyelin synthesis. {ECO:0000269|PubMed:17081983, ECO:0000269|PubMed:17591919, ECO:0000269|PubMed:18669648, ECO:0000269|PubMed:19005213}.
Similarity Contains 1 PH domain. {ECO:0000255|PROSITE- ProRule:PRU00145}.
Similarity Contains 1 START domain. {ECO:0000255|PROSITE- ProRule:PRU00197}.
Subcellular Location Cytoplasm. Golgi apparatus. Endoplasmic reticulum. Note=Preferentially localized to the Golgi apparatus.
Subunit Interacts with COL4A3. Interacts with VAPA and VAPB. Interaction with VAPB is less efficient than with VAPA. {ECO:0000269|PubMed:16895911}.
Tissue Specificity Widely expressed.