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Anti-PKHD1 Antibody (4M854) is a Mouse antibody targeting PKHD1. Anti-PKHD1 Antibody (4M854) can be used in WB,ICC,IHC,FCM.
Pack Size | Price | Availability | Quantity |
---|---|---|---|
50 μL | $296 | 7-10 days | |
100 μL | $427 | 7-10 days |
Description | Anti-PKHD1 Antibody (4M854) is a Mouse antibody targeting PKHD1. Anti-PKHD1 Antibody (4M854) can be used in WB,ICC,IHC,FCM. |
Synonyms | Tigmin, TIGM1, Polyductin, Polycystic kidney and hepatic disease 1 protein, PKHD1_HUMAN, PKHD 1, FPC, Fibrocystin, FCYT, ARPKD |
Reactivity | Human |
Verified Activity | 1. Western blot analysis of PKHD1 on mouse PKHD1 recombinant protein using anti-PKHD1 antibody at 1/1,000 dilution. 2. Immunohistochemical analysis of paraffin-embedded human esophageal cancer tissue using anti-PKHD1 antibody. Counter stained with hematoxylin. 3. 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. 4. 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). |
Application | |
Recommended Dose | WB: 1:500-2000; IHC: 1:50-200; ICC: 1:50-200; FCM: 1:50-100 |
Antibody Type | Monoclonal |
Host Species | Mouse |
Construction | Hybridoma Monoclonal Antibody |
Purification | ProA affinity purified |
Appearance | Liquid |
Formulation | 1*TBS (pH7.4), 1%BSA, 40%Glycerol. Preservative: 0.05% Sodium Azide. |
Research 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. |
Conjucates | Unconjugated |
Immunogen | Recombinant Protein |
Uniprot ID |
Molecular Weight | Theoretical: 445 kDa. |
Stability & Storage | Store at -20°C or -80°C for 12 months. Avoid repeated freeze-thaw cycles. |
Transport | Shipping with blue ice. |
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