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PKHD1 [A1-G12]
Catalog Number:OM627335
OM627335
Discount Price
OM627335-100ul
1~2 Week
100ul
OM627335-50ul
50ul
1~2 Week
Product Profile
Product Name PKHD1 [A1-G12]
Antibody Type Primary Antibodies
Antigen Alias 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
Product description 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.
Immunogen Recombinant protein
Key Feature
Clonality Monoclonal
Isotype IgG1
Host Species Mouse
Tested Applications WBICCIHCFC
WB:1:500-1:2,000
ICC:1:50-1:200
IHC:1:50-1:200
FC:1:50-1:100
Species Reactivity Human
Concentration 2mg/ml
Target Information
Alternative Names 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
Molecular Weight(MW) 445 kDa
Cellular Localization Membrane
Database Links
SwissProt ID P08F94
Application

Application

Fig1: Western blot analysis of PKHD1 on mouse PKHD1 recombinant protein using anti-PKHD1 antibody at 1/1,000 dilution.

Application

Fig2: ICC staining PKHD1 (green) and Actin filaments (red) in A431 cells. The nuclear counter stain is DAPI (blue). Cells were fixed in paraformaldehyde, permeabilised with 0.25% Triton X100/PBS.

Application

Fig3: Immunohistochemical analysis of paraffin-embedded human esophageal cancer tissue using anti-PKHD1 antibody. Counter stained with hematoxylin.

Application

Fig4: Flow cytometric analysis of Hela cells with PKHD1 antibody at 1/100 dilution (green) compared with an unlabelled control (cells without incubation with primary antibody; red).
Positive Control Mouse PKHD1 recombinant protein, A431, human esophageal cancer tissue, Hela.
Application Notes WB:1:500-1:2,000
ICC:1:50-1:200
IHC:1:50-1:200
FC:1:50-1:100
Additional Information
Form Liquid
Storage Instructions Store at +4℃ after thawing. Aliquot store at -20℃ or -80℃. Avoid repeated freeze / thaw cycles.
Storage Buffer 1*TBS (pH7.4), 1%BSA, 40%Glycerol. Preservative: 0.05% Sodium Azide.
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