84-1102-99　PKHD1 (Polycystic Kidney and Hepatic Disease 1, ARPKD, DKFZp686C01112, Fibrocystin, FCYT, FLJ46150, Polyductin, Tigmin, TIGM1) 100ul　P4210-70D

特徴

• 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: 1:100-1:2000Optimal 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.