Description
| Product Name: | Human COL4A3BP Recombinant Protein |
| Product Code: | RPPB3225 |
| Size: | 25µg |
| Species: | Human |
| Target: | COL4A3BP |
| Synonyms: | COL4A3BP, FLJ20597, Collagen type IV alpha-3-binding protein, GPBP, STARD11, CERT, HCERT, CERTL, Ceramide Transfer Protein, Goodpasture antigen-binding protein, StAR-related lipid transfer protein 11, START domain-containing protein 11. |
| Source: | Escherichia Coli |
| Physical Appearance: | Sterile filtered colorless solution. |
| Formulation: | COL4A3BP Human solution containing 20mM Tris HCL pH-8, 0.1M NaCl, & 10% glycerol. |
| Stability: | Store at 4°C if entire vial will be used within 2-4 weeks. Store, frozen at -20°C for longer periods of time. For long term storage it is recommended to add a carrier protein (0.1% HSA or BSA).Avoid multiple freeze-thaw cycles. |
| Purity: | Greater than 95.0% as determined by SDS-PAGE. |
| Amino Acid Sequence: | MRGSHHHHHH GMASMTGGQQ MGRDLYDDDD KDRWAGSMLH WPTSLPSGDA FSSVGTHRFV QKVEEMVQNH MTYSLQDVGG DANWQLVVEE GEMKVYRREV EENGIVLDPL KATHAVKGVT GHEVCNYFWN VDVRNDWETT IENFHVVETL ADNAIIIYQT HKRVWPASQR DVLYLSVIRK IPALTENDPE TWIVCNFSVD HDSAPLNNRC VRAKINVAMI CQTLVSPPEG NQEISRDNIL CKITYVANVN PGGWAPASVL RAVAKREYPK FLKRFTSYVQ EKTAGKPILF |
COL4A3BP is a kinase that particularly phosphorylates the N-terminal region of the non-collagenous domain of the alpha 3 chain of type IV collagen, recognized as the Goodpasture antigen that is the outcome of an autoimmune reaction directed at COL4A3BP. One isoform of COL4A3BP participates in ceramide intracellular transport.
COL4A3BP Recombinant Human produced in E.Coli is a single, non-glycosylated polypeptide chain containing 290 amino acids (347-598 a.a.) and having a molecular mass of 33.1 kDa. The COL4A3BP is fused to 38 amino acid His-Tag at N-terminus and purified by proprietary chromatographic techniques.
| UniProt Protein Function: | COL4A3BP: a protein with a lipid-binding domain that mediates intracellular trafficking of ceramide in a non-vesicular manner. Implicated in human autoimmune diseases via its association with the Goodpasture antigen. Phosphorylation on Ser-132 decreases the affinity toward phosphatidylinositol 4-phosphate at Golgi membranes and reduces ceramide transfer activity. GPBP has been reported to have Ser/Thr protein kinase activity towards the Goodpasture (GP) antigen, but lacks a conventional serine/threonine kinase domain. Two human isoforms of the human protein are produced by alternative splicing. |
| UniProt Protein Details: | Protein type:Kinase, protein; Protein kinase, Ser/Thr (non-receptor); EC 2.7.11.9; Lipid-binding; Protein kinase, atypical; ATYPICAL group; COL4A3BP family Chromosomal Location of Human Ortholog: 5q13.3 Cellular Component: nucleoplasm; Golgi apparatus; endoplasmic reticulum membrane; mitochondrion; perinuclear region of cytoplasm; cytosol Molecular Function:protein binding; protein kinase activity Biological Process: heart morphogenesis; endoplasmic reticulum organization and biogenesis; cell proliferation; sphingolipid metabolic process; muscle contraction; sphingolipid biosynthetic process; in utero embryonic development; cell morphogenesis; lipid homeostasis; immune response; signal transduction; ceramide metabolic process; protein amino acid phosphorylation Disease: Mental Retardation, Autosomal Dominant 34 |
| NCBI 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] |
| UniProt Code: | Q9Y5P4 |
| NCBI GenInfo Identifier: | 20978413 |
| NCBI Gene ID: | 10087 |
| NCBI Accession: | Q9Y5P4.1 |
| UniProt Secondary Accession: | Q9Y5P4,Q53YV1, Q53YV2, Q96Q85, Q96Q88, Q9H2S7, Q9H2S8 A8K7S2, B3KUB7, |
| UniProt Related Accession: | Q9Y5P4 |
| Molecular Weight: | 83,708 Da |
| NCBI Full Name: | Collagen type IV alpha-3-binding protein |
| NCBI Synonym Full Names: | collagen, type IV, alpha 3 (Goodpasture antigen) binding protein |
| NCBI Official Symbol: | COL4A3BP�� |
| NCBI Official Synonym Symbols: | CERT; GPBP; CERTL; STARD11�� |
| NCBI Protein Information: | 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 |
| UniProt Protein Name: | Collagen type IV alpha-3-binding protein |
| UniProt Synonym Protein Names: | Ceramide transfer protein; hCERT; Goodpasture antigen-binding protein; GPBP; START domain-containing protein 11; StARD11; StAR-related lipid transfer protein 11 |
| Protein Family: | Collagen type IV alpha-3-binding protein |
| UniProt Gene Name: | COL4A3BP�� |
| UniProt Entry Name: | C43BP_HUMAN |
