Anti-PKHD1 antibody [A1-G12]

Anti-PKHD1 antibody [A1-G12]

• 概述

• 产品描述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.
• 产品名称Anti-PKHD1 antibody [A1-G12]
• 分子量445 kDa
• 种属反应性Human
• 验证应用WB,ICC,IHC-P,FC
• 抗体类型小鼠单抗
• 免疫原Recombinant protein
• 偶联Non-conjugated

• 性能

• 形态Liquid
• 浓度2 mg/mL.
• 存放说明Store at +4℃ after thawing. Aliquot store at -20℃ or -80℃. Avoid repeated freeze / thaw cycles.
• 存储缓冲液1*TBS (pH7.4), 1%BSA, 40%Glycerol. Preservative: 0.05% Sodium Azide.
• 亚型IgG1
• 纯化方式Protein A purified.
• 亚细胞定位Membrane
• 其它名称
  • ARPKD antibody
  • FCYT antibody
  • Fibrocystin antibody
  more

• 应用

  WB: 1:500-1:2,000
  ICC: 1:50-1:200
  IHC-P: 1:50-1:200
  FC: 1:50-1:100

•  

  

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

   

  

  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.

   

  

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

   

  

  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).

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