Product Name :
PKHD1 Mouse Monoclonal Antibody [A1-G12]
Predicted band size :
Observed band size :
Synonyms:
ARPKD antibody FCYT antibody Fibrocystin antibody FPC antibody PKHD1 antibody PKHD1_HUMAN antibody Polycystic kidney and hepatic disease 1 protein antibody Polyductin antibody TIGM1 antibody Tigmin antibody
Function :
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.36294-24-3 site 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.1086423-62-2 Formula
Antibody Type:
Mouse Monoclonal Antibody
Immunogen :
Purified recombinant fragment of mouse PKHD1 (AA: 3878-4060) expressed in E. Coli.
Species reactivity:
Human
Verify application:
ICC, IHC-P, FC
Coupling :
unconjugated
Clone number:
A1-G12
RRID:
Form :
Liquid
Concentration :
2ug/ul
Storage instructions:
4℃; -20℃ for long term storage.PMID:33427019
Storage buffer solution:
Purified antibody in PBS with 0.05% sodium azide.
Subtype:
IgG1
Purification method :
Protein A affinity purified.
ELTSA :
Molecular weight :
445 kDa
Positive control :
Mouse PKHD1 recombinant protein, A431, human esophageal cancer tissue, Hela.