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

Size

£594.00

SKU:: P4210-70D
Purity:: Purified by immunoaffinity chromatography.
Storage Conditions:: -20°C
Supplier:: United States Biological
Host:: Rabbit
Immunogen:: Synthetic peptide corresponding to human PKHD1 (within residues 3800-3900). Species sequence homology: mouse (86%). Cellular Localization: Membrane; single-pass type I membrane.
Format:: Supplied as a liquid in PBS, 0.05% sodium azide, 30% glycerol.
Extra Details:: 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. Applications: Suitable for use in ELISA and Immunocytochemistry/Immunofluorescence. Other applications not tested. Recommended Dilution: Peptide ELISA: 1:100-1:2000 Optimal dilutions to be determined by the researcher. Storage and Stability: May be stored at 4°C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20°C. Aliquots are stable for 12 months after receipt. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.

Shipping Conditions:: Blue Ice