PKHD1 Antibody [HRP]

Product Discontinued

PKHD1 Antibody [HRP] Summary

• Immunogen: A synthetic peptide derived from the human PKHD1 protein (within residues 3300-3400). [UniProt# Q8TCZ9]
• Localization: Membrane; single-pass type I membrane.
• Isotype: IgG
• Clonality: Polyclonal
• Host: Rabbit
• Gene: PKHD1
• Purity: Immunogen affinity purified

Applications/Dilutions

• Dilutions:
  • Immunocytochemistry/ Immunofluorescence
  • Peptide ELISA
• Application Notes: This PKHD1 antibody is useful for peptide ELISA.
• Readout System:
  • NovaLume Plus Chemiluminescent Reagent LUM-555

Reactivity Notes

Immunogen sequence has 83% identity to mouse. Customer review on mouse kidney collecting duct cell line.

Packaging, Storage & Formulations

• Storage: Store at 4C in the dark.
• Buffer: PBS
• Preservative: No Preservative
• Purity: Immunogen affinity purified

Alternate Names for PKHD1 Antibody [HRP]

• polycystic kidney and hepatic disease 1 (autosomal recessive)

Background

PKHD1 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 PKHD1. 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. PKHD1 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 PKHD1 interacts with calcium modulating cyclophilin ligand (CAML), a protein implicated in calcium signaling. PKHD1 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 result in autosomal recessive polycystic kidney disease (ARPKD), a severe form of polycystic kidney disease characterized by enlarged kidneys and congenital hepatic fibrosis.

Limitations

This product is for research use only and is not approved for use in humans or in clinical diagnosis. Primary Antibodies are guaranteed for 1 year from date of receipt.

Bioinformatics

• Gene Symbol: PKHD1