| 存储条件 |
|---|
| 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 |
| 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 |
| 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 |
| SwissProt ID | P08F94 |
|---|
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 |
| 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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