PKHD1 Antibody

Product Discontinued

PKHD1 Antibody Summary

• Immunogen: A synthetic peptide derived from the human PKHD1 protein (within residues 450-550). [UniProt# Q8TCZ9]
• Localization: Membrane; single-pass type I membrane.
• Isotype: IgG
• Clonality: Polyclonal
• Host: Rabbit
• Gene: PKHD1
• Purity: Immunogen affinity purified

Applications/Dilutions

• Dilutions:
  • Peptide ELISA 1:100-1:2000
• Application Notes: This PKHD1 antibody is useful for peptide ELISA.

Reactivity Notes

Human. Immunogen sequence has 88% identity to mouse.

Packaging, Storage & Formulations

• Storage: Aliquot and store at -20C or -80C. Avoid freeze-thaw cycles.
• Buffer: PBS and 30% Glycerol
• Preservative: 0.05% Sodium Azide
• Concentration: 0.2 mg/ml
• Purity: Immunogen affinity purified

Notes

Please note, this antibody is considered Innovators Grade. Innovators Grade antibodies are generally unvalidated and require additional characterization for most new species/applications. Novus has made these antibodies available through our Innovators Reward program. Complete an online review with image, detailing your positive or negative results. In return, you receive a discount voucher for 100% of the purchase price of the reviewed product. Please contact us at innovators@novusbio.com for more details.

Alternate Names for PKHD1 Antibody

• polycystic kidney and hepatic disease 1 (autosomal recessive)

Background

PKHD1 protein (polycystic kidney and hepatic disease 1 protein) is encoded by PKHD1 gene whose mutations have been established as underlying cause of autosomal recessive polycystic kidney disease (ARPKD), a rare genetic disorder characterized mainly by cystic kidneys in the developing fetus in utero and post-natal pulmonary insufficiency as well as renal manifestations, abnormal biliary development with dilated bile ductules and peribiliary fibrosis, collectively termed congenital hepatic fibrosis (CHF). PKHD1 is a type I membrane protein with a large extracellular N-terminal domain, a single transmembrane segment, and a short cytoplasmic C-terminus. PKHD1 is a large protein with predicted molecular weight of 447 kD and is predominantly expressed in kidneys (the cortical and medullary collecting ducts), pancreas, liver etc. and in addition to other locations in the cells, it is localized on primary cilium, hair-like organelle present on cellular surfaces. PKHD1 is essential for correct bipolar cell division through the regulation of centrosome duplication and mitotic spindle assembly. Moreover, it has been suggested to act as a receptor that plays a role in collecting duct/biliary differentiation and lack of PKHD1 results in stunted primary cilia which have been proposed to underlie the pathogenesis of ARPKD.

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
• Entrez:
  • 5314(Human)
• Uniprot:
  • P08F94(Human)