PKHD1 Antibody

Catalog Number:

48398

Amount:

100μg

Price:

$529

Swiss-Prot No:

Swiss-Prot#:P08F94

Form of Antibody:

1*TBS (pH7.4), 1%BSA, 40%Glycerol. Preservative: 0.05% Sodium Azide.

Storage/Stability:

Immunogen:

Recombinant protein

Purification:

ProA affinity purified

Specificity/Sensitivity:

Applications:

WB, ICC, IHC, FC

Background:

May be required for correct bipolar cell division through the regulation of centrosome duplication and mitotic spindle assembly. May be a receptor protein that acts in collecting-duct and biliary differentiation. Defects in PKHD1 are the cause of polycystic kidney disease autosomal recessive (ARPKD). ARPKD is a severe form of polycystic kidney disease affecting the kidneys and the hepatic biliary tract. The clinical spectrum is widely variable, with most cases presenting during infancy. The fetal phenotypic features classically include enlarged and echogenic kidneys, as well as oligohydramnios secondary to a poor urine output. Up to 50% of the affected neonates die shortly after birth, as a result of severe pulmonary hypoplasia and secondary respiratory insufficiency. In the subset that survives the perinatal period, morbidity and mortality are mainly related to severe systemic hypertension, renal insufficiency, and portal hypertension due to portal-tract fibrosis.

References:

appl_detail:

WB: 1:500-1:2,000
IHC: 1:50-1:200
ICC: 1:50-1:200

FC: 1:50-1:100

Datasheet PDF