COL4A3BP blocking peptide
COL4A3BP Blocking Peptide

NCBI and Uniprot Product Information
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