NCBI Description
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 Description
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.
Protein type: Protein kinase, Ser/Thr (non-receptor); EC 2.7.11.9; Lipid-binding; Kinase, protein; 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; sphingolipid metabolic process; sphingolipid biosynthetic process; in utero embryonic development; cell morphogenesis; lipid homeostasis; signal transduction; protein amino acid phosphorylation; cell proliferation; muscle contraction; immune response; ceramide metabolic process
Disease: Mental Retardation, Autosomal Dominant 34
Product Notes
The GPBP col4a3bp (Catalog #
AAA8306340) is an Antibody produced from Rabbit and is intended for research purposes only. The product is available for immediate purchase. The Anti-GPBP Antibody reacts with Human, Mouse, Rat and may cross-react with other species as described in the data sheet. AAA Biotech's GPBP can be used in a range of immunoassay formats including, but not limited to, Western Blot (WB). WB: 1:500-1:2000. Researchers should empirically determine the suitability of the GPBP col4a3bp for an application not listed in the data sheet. Researchers commonly develop new applications and it is an integral, important part of the investigative research process.
It is sometimes possible for the material contained within the vial of
"GPBP, Polyclonal Antibody" to become dispersed throughout the inside of
the vial, particularly around the seal of said vial, during shipment and storage. We always
suggest centrifuging these vials
to consolidate all of the liquid away from the lid and to the bottom of the vial prior to
opening. Please be advised that
certain products may require dry ice for shipping and that, if this is the case, an
additional dry ice fee may also be
required.
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absolutely not suitable for use in any
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product from AAA Biotech, you
are explicitly certifying that said products will be properly tested and used in line with
industry standard. AAA Biotech
and its authorized distribution partners reserve the right to refuse to fulfill any order if
we have any indication that a
purchaser may be intending to use a product outside of our accepted criteria.
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