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PKHD1 (Polycystic Kidney and Hepatic Disease 1, ARPKD, DKFZp686C01112, Fibrocystin, FCYT, FLJ46150, Polyductin, Tigmin, TIGM1)

Cat no: P4210-70B

PKHD1 (Polycystic Kidney and Hepatic Disease 1, ARPKD, DKFZp686C01112, Fibrocystin, FCYT, FLJ46150, Polyductin, Tigmin, TIGM1)

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.\n\nCellular Localization: Membrane; single-pass type I membrane.\n\nApplications: \nSuitable for use in ELISA. Other applications not tested.\n\nRecommended Dilution:\nOptimal dilutions to be determined by the researcher.\n\nStorage and Stability:\nMay be stored at 4 degrees C for short-term only. For long-term storage and to avoid repeated freezing and thawing, aliquot and add glycerol (40-50%) Freeze at -20 degrees C or colder. Aliquots are stable for at least 12 months at -20 degrees C. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap. Further dilutions can be made in assay buffer.

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SPECIFICATIONS

Catalog Number

P4210-70B

Size

100ul

Applications

ELISA

Hosts

Rabbit

Reactivities

Hum

Form

Supplied as a liquid in PBS, pH 7.2, 30% glycerol.

P Type

Pab

Purity

Purified by peptide immunoaffinity chromatography.

Additional Info

The specificity of this antibody is unknown. Species Crossreactivity: Species reactivity for this antibody has not been determined.

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