Fibrocystin / PKHD1 antibody

Principal name

Fibrocystin / PKHD1 antibody

Alternative names for Fibrocystin / PKHD1 antibody

FCYT, TIGM1, Tigmin, Polyductin, Polycystic kidney and hepatic disease 1 protein

SwissProt ID

P08F94 (Human), Q8TCZ9 (Human)

Gene ID

5314 (PKHD1)

Available reactivities

Hu (Human), Ms (Mouse)

Available hosts

Rabbit, Mouse

Available applications

Enzyme Immunoassay (E)

Background of Fibrocystin / PKHD1 antibody

BOOT CAMP BACKGROUND: We know how important your research is. For this reason Novus Biologicals offers Boot Camp antibodies to accelerate your discovery based research. Boot Camp antibodies are directed to epitopes on proteins of scientific merit to which there is a scarcity of quality antibodies. Boot Camp antibodies have all been proven by ELISA to exhibit high titers to synthetic peptides that represent their cognate epitopes. However, Novus Biologicals issues no guarantee that Boot Camp antibodies will react with the target protein. _____________________________________________ GENERAL BACKGROUND: Fibrocystin is a type I membrane protein that undergoes regulated proteolysis. Many proteolytic cleavages occur on the ectodomain whereas at least one cleavage occurs on the cytoplasmic portion of fibrocystin. The later generates a C-terminal intracellular fragment that localizes to the nucleus. This proteolysis requires activation of protein kinase C (PKC) and release of intracellular calcium. Fibrocystin is expressed in the cilia of the bile duct epithelium and leads to abnormalities in the rubric of the ductal plate malformation. The intracellular C-terminus of Fibrocystin interacts with calcium modulating cyclophilin ligand (CAML), a protein implicated in calcium signaling. Fibrocystin may participate in the mediation of intracellular calcium in the distal nephron in a manner similar to PKD1 and PKD2. Mutations in the PKHD1 gene, which encodes Fibrocystin, result in autosomal recessive polycystic kidney disease (ARPKD), a severe form of polycystic kidney disease characterized by enlarged kidneys and congenital hepatic fibrosis.

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