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Rabbit Anti-COL4A3BP/FITC Conjugated antibody
background:
COL4A3BP is 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. Two transcripts exist for this gene.
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.
Subunit:
Interacts with COL4A3. Interacts with VAPA and VAPB. Interaction with VAPB is less efficient than with VAPA.
Subcellular Location:
Cytoplasm. Golgi apparatus. Endoplasmic reticulum.
Tissue Specificity:
Widely expressed.
Post-translational modifications:
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.
Similarity:
Contains 1 PH domain.
Contains 1 START domain.
Database links:
Entrez Gene: 10087 Human
Entrez Gene: 13618 Mouse
Entrez Gene: 365652 Rat
Omim: 604677 Human
SwissProt: Q9GKI7 Cow
SwissProt: Q9Y5P4 Human
SwissProt: Q9EQG9 Mouse
Unigene: 270437 Human
Unigene: 24125 Mouse
Unigene: 105704 Rat
Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
COL4A3BP蛋白目前,主要用于Goodpasture综合征等方面的研究。
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