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
P4210-70C.100 100 µl - -

3 - 19 business days

623.00€
 
PKHD1 protein (polycystic kidney and hepatic disease 1 protein) is encoded by PKHD1 gene whose... more
Product information "Anti-PKHD1 (Polycystic Kidney and Hepatic Disease 1, ARPKD, DKFZp686C01112, Fibrocystin, FCYT, FLJ46"
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. Cellular Localization: Membrane, single-pass type I membrane. , Applications: , Suitable for use in Peptide ELISA and Immunocytochemistry/Immunofluorescence. 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: P4210-70C

Properties

Application: ELISA
Antibody Type: Polyclonal
Conjugate: No
Host: Rabbit
Reactivity: Human
Immunogen: A synthetic peptide derived from the human PKHD1 protein (within residues 3300-3400). Homology: 100% homology with human protein. 83% 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!
You will get a certificate here
or to request a certificate of analysis.
Read, write and discuss reviews... more
Customer review for "Anti-PKHD1 (Polycystic Kidney and Hepatic Disease 1, ARPKD, DKFZp686C01112, Fibrocystin, FCYT, FLJ46"
Write a review
or to review a product.
Viewed