Key Product Details

Species Reactivity

Human, Mouse

Applications

Immunocytochemistry/ Immunofluorescence, Peptide ELISA

Label

Unconjugated

Antibody Source

Polyclonal Rabbit IgG

Concentration

0.2 mg/ml

View all PKHD1 Antibodies »

Product Specifications

Immunogen

A synthetic peptide derived from the human PKHD1 protein (within residues 3800-3900). [UniProt# Q8TCZ9]

Reactivity Notes

Immunogen sequence has 86% identity to mouse.

Localization

Membrane; single-pass type I membrane.

Clonality

Polyclonal

Host

Rabbit

Isotype

IgG

Applications for PKHD1 Antibody

Application
Recommended Usage

Peptide ELISA

1:100-1:2000

Formulation, Preparation, and Storage

Purification

Immunogen affinity purified

Formulation

PBS and 30% Glycerol

Preservative

0.05% Sodium Azide

Concentration

0.2 mg/ml

Shipping

The product is shipped with polar packs. Upon receipt, store it immediately at the temperature recommended below.

Stability & Storage

Aliquot and store at -20C or -80C. Avoid freeze-thaw cycles.

Background: PKHD1

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.

Alternate Names

polycystic kidney and hepatic disease 1 (autosomal recessive)

Entrez Gene IDs

5314 (Human)

Gene Symbol

PKHD1

UniProt

P08F94 (Human)

Additional PKHD1 Products

Product Documents for PKHD1 Antibody

Product Datasheets

Certificate of Analysis

To download a Certificate of Analysis, please enter a lot number in the search box below.

Download SDS

Product Specific Notices for PKHD1 Antibody

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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