PKHD1 Antibody

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Product Details

Summary
Reactivity Hu, MuSpecies Glossary
Applications ELISA, ICC/IF
Clonality
Polyclonal
Host
Rabbit
Conjugate
Unconjugated
Concentration
0.2 mg/ml

Order Details

PKHD1 Antibody 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
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Test in a species/application not listed above to receive a full credit towards a future purchase.

Applications/Dilutions

Dilutions
  • Immunocytochemistry/ Immunofluorescence
  • Peptide ELISA 1:100-1:2000
Application Notes
This PKHD1 antibody is useful for peptide ELISA. Positive results have also been obtained by IF of mouse kidney collecting duct cell line.

Reactivity Notes

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

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

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.

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Product General Protocols

Find general support by application which include: protocols, troubleshooting, illustrated assays, videos and webinars.

Video Protocols

ICC/IF Video Protocol

FAQs for PKHD1 Antibody (BC110-60479) (0)

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Bioinformatics

Gene Symbol PKHD1
Entrez
Uniprot