Gene/Proteome Database (LMPD)
LMPD ID
LMP005071
Gene ID
Species
Homo sapiens (Human)
Gene Name
collagen, type IV, alpha 3 (Goodpasture antigen) binding protein
Gene Symbol
Synonyms
CERT; CERTL; GPBP; STARD11
Alternate Names
collagen type IV alpha-3-binding protein; hCERT; ceramide transporter; ceramide transfer protein; lipid-transfer protein CERTL; StAR-related lipid transfer (START) domain containing 11
Chromosome
5
Map Location
5q13.3
Summary
This gene encodes a kinase that specifically phosphorylates the N-terminal region of the non-collagenous domain of the alpha 3 chain of type IV collagen, known as the Goodpasture antigen. Goodpasture disease is the result of an autoimmune response directed at this antigen. One isoform of this protein is also involved in ceramide intracellular transport. Three transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Jul 2008]
Orthologs
Proteins
| collagen type IV alpha-3-binding protein isoform 1 | |
|---|---|
| Refseq ID | NP_005704 |
| Protein GI | 5031717 |
| UniProt ID | Q9Y5P4 |
| mRNA ID | NM_005713 |
| Length | 624 |
| RefSeq Status | REVIEWED |
| MSDNQSWNSSGSEEDPETESGPPVERCGVLSKWTNYIHGWQDRWVVLKNNALSYYKSEDETEYGCRGSICLSKAVITPHDFDECRFDISVNDSVWYLRAQDPDHRQQWIDAIEQHKTESGYGSESSLRRHGSMVSLVSGASGYSATSTSSFKKGHSLREKLAEMETFRDILCRQVDTLQKYFDACADAVSKDELQRDKVVEDDEDDFPTTRSDGDFLHSTNGNKEKLFPHVTPKGINGIDFKGEAITFKATTAGILATLSHCIELMVKREDSWQKRLDKETEKKRRTEEAYKNAMTELKKKSHFGGPDYEEGPNSLINEEEFFDAVEAALDRQDKIEEQSQSEKVRLHWPTSLPSGDAFSSVGTHRFVQKPYSRSSSMSSIDLVSASDDVHRFSSQVEEMVQNHMTYSLQDVGGDANWQLVVEEGEMKVYRREVEENGIVLDPLKATHAVKGVTGHEVCNYFWNVDVRNDWETTIENFHVVETLADNAIIIYQTHKRVWPASQRDVLYLSVIRKIPALTENDPETWIVCNFSVDHDSAPLNNRCVRAKINVAMICQTLVSPPEGNQEISRDNILCKITYVANVNPGGWAPASVLRAVAKREYPKFLKRFTSYVQEKTAGKPILF | |
| collagen type IV alpha-3-binding protein isoform 2 | |
|---|---|
| Refseq ID | NP_112729 |
| Protein GI | 14165452 |
| UniProt ID | Q9Y5P4 |
| mRNA ID | NM_031361 |
| Length | 598 |
| RefSeq Status | REVIEWED |
| MSDNQSWNSSGSEEDPETESGPPVERCGVLSKWTNYIHGWQDRWVVLKNNALSYYKSEDETEYGCRGSICLSKAVITPHDFDECRFDISVNDSVWYLRAQDPDHRQQWIDAIEQHKTESGYGSESSLRRHGSMVSLVSGASGYSATSTSSFKKGHSLREKLAEMETFRDILCRQVDTLQKYFDACADAVSKDELQRDKVVEDDEDDFPTTRSDGDFLHSTNGNKEKLFPHVTPKGINGIDFKGEAITFKATTAGILATLSHCIELMVKREDSWQKRLDKETEKKRRTEEAYKNAMTELKKKSHFGGPDYEEGPNSLINEEEFFDAVEAALDRQDKIEEQSQSEKVRLHWPTSLPSGDAFSSVGTHRFVQKVEEMVQNHMTYSLQDVGGDANWQLVVEEGEMKVYRREVEENGIVLDPLKATHAVKGVTGHEVCNYFWNVDVRNDWETTIENFHVVETLADNAIIIYQTHKRVWPASQRDVLYLSVIRKIPALTENDPETWIVCNFSVDHDSAPLNNRCVRAKINVAMICQTLVSPPEGNQEISRDNILCKITYVANVNPGGWAPASVLRAVAKREYPKFLKRFTSYVQEKTAGKPILF | |
| collagen type IV alpha-3-binding protein isoform 3 | |
|---|---|
| Refseq ID | NP_001123577 |
| Protein GI | 194294543 |
| UniProt ID | Q9Y5P4 |
| mRNA ID | NM_001130105 |
| Length | 752 |
| RefSeq Status | REVIEWED |
| MQHSCIPTPPSPFSAPPAFLPVVTRESRRGLSSGGSAGRNAGVTATAAAADGWKGRLPSPLVLLPRSARCQARRRRGGRTSSLLLLPPTPERALFASPSPDPSPRGLGASSGAAEGAGAGLLLGCRASMSDNQSWNSSGSEEDPETESGPPVERCGVLSKWTNYIHGWQDRWVVLKNNALSYYKSEDETEYGCRGSICLSKAVITPHDFDECRFDISVNDSVWYLRAQDPDHRQQWIDAIEQHKTESGYGSESSLRRHGSMVSLVSGASGYSATSTSSFKKGHSLREKLAEMETFRDILCRQVDTLQKYFDACADAVSKDELQRDKVVEDDEDDFPTTRSDGDFLHSTNGNKEKLFPHVTPKGINGIDFKGEAITFKATTAGILATLSHCIELMVKREDSWQKRLDKETEKKRRTEEAYKNAMTELKKKSHFGGPDYEEGPNSLINEEEFFDAVEAALDRQDKIEEQSQSEKVRLHWPTSLPSGDAFSSVGTHRFVQKPYSRSSSMSSIDLVSASDDVHRFSSQVEEMVQNHMTYSLQDVGGDANWQLVVEEGEMKVYRREVEENGIVLDPLKATHAVKGVTGHEVCNYFWNVDVRNDWETTIENFHVVETLADNAIIIYQTHKRVWPASQRDVLYLSVIRKIPALTENDPETWIVCNFSVDHDSAPLNNRCVRAKINVAMICQTLVSPPEGNQEISRDNILCKITYVANVNPGGWAPASVLRAVAKREYPKFLKRFTSYVQEKTAGKPILF | |
Gene Information
Entrez Gene ID
Gene Name
collagen, type IV, alpha 3 (Goodpasture antigen) binding protein
Gene Symbol
Species
Homo sapiens
Gene Ontology (GO Annotations)
| GO ID | Source | Type | Description |
|---|---|---|---|
| GO:0005829 | TAS:Reactome | C | cytosol |
| GO:0005789 | TAS:Reactome | C | endoplasmic reticulum membrane |
| GO:0005794 | IDA:UniProtKB | C | Golgi apparatus |
| GO:0005739 | IEA:Ensembl | C | mitochondrion |
| GO:0005634 | IDA:HPA | C | nucleus |
| GO:0097001 | IDA:UniProtKB | F | ceramide binding |
| GO:0035620 | IDA:UniProtKB | F | ceramide transporter activity |
| GO:0070273 | IDA:UniProtKB | F | phosphatidylinositol-4-phosphate binding |
| GO:0004672 | TAS:ProtInc | F | protein kinase activity |
| GO:0000902 | IEA:Ensembl | P | cell morphogenesis |
| GO:0008283 | IEA:Ensembl | P | cell proliferation |
| GO:0006672 | IEA:Ensembl | P | ceramide metabolic process |
| GO:0007029 | IEA:Ensembl | P | endoplasmic reticulum organization |
| GO:0035621 | IMP:UniProtKB | P | ER to Golgi ceramide transport |
| GO:0003007 | IEA:Ensembl | P | heart morphogenesis |
| GO:0006955 | NAS:UniProtKB | P | immune response |
| GO:0001701 | IEA:Ensembl | P | in utero embryonic development |
| GO:0055088 | IEA:Ensembl | P | lipid homeostasis |
| GO:0070584 | IEA:Ensembl | P | mitochondrion morphogenesis |
| GO:0006936 | IEA:Ensembl | P | muscle contraction |
| GO:0006468 | TAS:ProtInc | P | protein phosphorylation |
| GO:0034976 | IEA:Ensembl | P | response to endoplasmic reticulum stress |
| GO:0007165 | IEA:Ensembl | P | signal transduction |
| GO:0044281 | TAS:Reactome | P | small molecule metabolic process |
| GO:0030148 | TAS:Reactome | P | sphingolipid biosynthetic process |
| GO:0006665 | TAS:Reactome | P | sphingolipid metabolic process |
REACTOME Pathway Links
| REACTOME Pathway ID | Description |
|---|---|
| REACT_111217 | Metabolism |
| REACT_22258 | Metabolism of lipids and lipoproteins |
| REACT_115810 | Sphingolipid de novo biosynthesis |
| REACT_19323 | Sphingolipid metabolism |
Domain Information
UniProt Annotations
Entry Information
Gene Name
collagen, type IV, alpha 3 (Goodpasture antigen) binding protein
Protein Entry
C43BP_HUMAN
UniProt ID
Species
Human
Comments
| Comment Type | Description |
|---|---|
| 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). |
| Domain | The FFAT motif is required for interaction with VAPA and VAPB. |
| Domain | The PH domain targets the Golgi apparatus. |
| Domain | The START domain recognizes ceramides and diacylglycerol lipids, interacts with membranes, and mediates the intermembrane transfer of ceramides and diacylglycerol lipids. |
| 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 |
| 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 |
| Similarity | Contains 1 PH domain. {ECO |
| Similarity | Contains 1 START domain. {ECO |
| 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. |
| Tissue Specificity | Widely expressed. |
Identical and Related Proteins
Unique RefSeq proteins for LMP005071 (as displayed in Record Overview)
| Protein GI | Database | Accession | Length | Protein Name |
|---|---|---|---|---|
| 5031717 | RefSeq | NP_005704 | 624 | collagen type IV alpha-3-binding protein isoform 1 |
| 14165452 | RefSeq | NP_112729 | 598 | collagen type IV alpha-3-binding protein isoform 2 |
| 194294543 | RefSeq | NP_001123577 | 752 | collagen type IV alpha-3-binding protein isoform 3 |
Identical Sequences to LMP005071 proteins
| Reference | Database | Accession | Length | Protein Name |
|---|---|---|---|---|
| GI:194294543 | DBBJ | BAG53379.1 | 752 | unnamed protein product [Homo sapiens] |
| GI:194294543 | GenBank | EAW95760.1 | 752 | collagen, type IV, alpha 3 (Goodpasture antigen) binding protein, isoform CRA_c [Homo sapiens] |
| GI:194294543 | GenBank | ABP28966.1 | 752 | Sequence 3981 from patent US 7193069 |
| GI:14165452 | GenBank | AFO06886.1 | 598 | Sequence 11 from patent US 8221999 |
| GI:5031717 | GenBank | AFO06887.1 | 624 | Sequence 13 from patent US 8221999 |
| GI:5031717 | GenBank | AFO14920.1 | 624 | Sequence 404 from patent US 8211634 |
| GI:5031717 | GenBank | AHD70367.1 | 624 | Sequence 3042 from patent US 8586006 |
| GI:14165452 | GenBank | AHD70368.1 | 598 | Sequence 3043 from patent US 8586006 |
| GI:14165452 | GenBank | AIC50498.1 | 598 | COL4A3BP, partial [synthetic construct] |
| GI:14165452 | GenBank | AIC62668.1 | 598 | COL4A3BP, partial [synthetic construct] |
| GI:194294543 | RefSeq | XP_526893.2 | 752 | PREDICTED: collagen type IV alpha-3-binding protein isoform X3 [Pan troglodytes] |
| GI:194294543 | RefSeq | XP_003810502.1 | 752 | PREDICTED: collagen type IV alpha-3-binding protein isoform X2 [Pan paniscus] |
| GI:5031717 | RefSeq | XP_006714576.1 | 624 | PREDICTED: collagen type IV alpha-3-binding protein isoform X1 [Homo sapiens] |
| GI:14165452 | RefSeq | XP_008961064.1 | 598 | PREDICTED: collagen type IV alpha-3-binding protein isoform X1 [Pan paniscus] |
| GI:5031717 | RefSeq | XP_009447279.1 | 624 | PREDICTED: collagen type IV alpha-3-binding protein isoform X1 [Pan troglodytes] |
| GI:5031717 | RefSeq | XP_009447281.1 | 624 | PREDICTED: collagen type IV alpha-3-binding protein isoform X1 [Pan troglodytes] |
| GI:14165452 | RefSeq | XP_009447282.1 | 598 | PREDICTED: collagen type IV alpha-3-binding protein isoform X2 [Pan troglodytes] |
Related Sequences to LMP005071 proteins
| Reference | Database | Accession | Length | Protein Name |
|---|---|---|---|---|
| GI:5031717 | EMBL | CAH23471.1 | 727 | unnamed protein product [Homo sapiens] |
| GI:14165452 | EMBL | CAH23472.1 | 611 | unnamed protein product, partial [synthetic construct] |
| GI:14165452 | EMBL | CAH23388.1 | 629 | unnamed protein product, partial [synthetic construct] |
| GI:5031717 | EMBL | CAH23389.1 | 678 | unnamed protein product, partial [synthetic construct] |
| GI:14165452 | EMBL | CAH23390.1 | 652 | unnamed protein product, partial [synthetic construct] |
| GI:5031717 | EMBL | CAH23393.1 | 726 | unnamed protein product, partial [synthetic construct] |
| GI:14165452 | GenBank | ABZ68470.1 | 611 | Sequence 12 from patent US 7326768 |
| GI:14165452 | GenBank | ABZ68472.1 | 629 | Sequence 16 from patent US 7326768 |
| GI:5031717 | GenBank | ABZ68473.1 | 678 | Sequence 18 from patent US 7326768 |
| GI:14165452 | GenBank | ABZ68474.1 | 652 | Sequence 20 from patent US 7326768 |
| GI:5031717 | GenBank | ABZ68475.1 | 702 | Sequence 22 from patent US 7326768 |
| GI:5031717 | GenBank | ABZ68477.1 | 726 | Sequence 26 from patent US 7326768 |
| GI:194294543 | GenBank | EHH54337.1 | 752 | Ceramide transfer protein [Macaca fascicularis] |
| GI:194294543 | RefSeq | XP_003266123.1 | 747 | PREDICTED: collagen type IV alpha-3-binding protein [Nomascus leucogenys] |
| GI:194294543 | RefSeq | XP_004058755.1 | 753 | PREDICTED: collagen type IV alpha-3-binding protein [Gorilla gorilla gorilla] |
| GI:194294543 | RefSeq | XP_005557241.1 | 752 | PREDICTED: collagen type IV alpha-3-binding protein isoform X1 [Macaca fascicularis] |
| GI:194294543 | RefSeq | XP_005557242.1 | 752 | PREDICTED: collagen type IV alpha-3-binding protein isoform X2 [Macaca fascicularis] |
| GI:194294543 | RefSeq | XP_007973903.1 | 752 | PREDICTED: collagen type IV alpha-3-binding protein isoform X3 [Chlorocebus sabaeus] |