Anti-PKHD1 (Polycystic Kidney and Hepatic Disease 1, ARPKD, DKFZp686C01112, Fibrocystin, FCYT, FLJ46

Anti-PKHD1 (Polycystic Kidney and Hepatic Disease 1, ARPKD, DKFZp686C01112, Fibrocystin, FCYT, FLJ46
Item number Size Datasheet Manual SDS Delivery time Quantity Price
P4210-70A.100 100 µl - -

3 - 19 business days*

623.00€
 
Fibrocystin is a type I membrane protein that undergoes regulated proteolysis. Many proteolytic... more
Product information "Anti-PKHD1 (Polycystic Kidney and Hepatic Disease 1, ARPKD, DKFZp686C01112, Fibrocystin, FCYT, FLJ46"
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 Peptide ELISA. 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.
Supplier: United States Biological
Supplier-Nr: P4210-70A

Properties

Application: ELISA
Antibody Type: Polyclonal
Conjugate: No
Host: Rabbit
Reactivity: Human
Immunogen: A synthetic peptide derived from the human PKHD1 protein (within residues 100-200). Homology: 100% homology with human protein.93% homology with mouse protein.
Purity: Purified by peptide immunoaffinity chromatography.
Format: Affinity Purified

Database Information

Handling & Safety

Storage: -20°C (-20°C Glycerol)
Shipping: +4°C (International: +4°C)
Caution
Our products are for laboratory research use only: Not for administration to humans!
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