概述

  • 产品名称Anti-PKHD1抗体
    参阅全部 PKHD1 一抗
  • 描述
    兔多克隆抗体to PKHD1
  • 经测试应用适用于: IHC-Pmore details
  • 种属反应性
    与反应: Human
  • 免疫原

    antigen sequence: KTTTVNYVRD TLSNPRGWMA LLLDQETYSL QSENLWINRS LQYSATFDNF APGNYLLLVH TDLPPYPDIL LRCGSRVGLS FPFLPSPGQN Q, corresponding to amino acids 2596-2686 of Human PKHD1.

  • 阳性对照
    • Human liver tissue.

性能

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应用

Our Abpromise guarantee covers the use of ab122160 in the following tested applications.

The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.

应用 Ab评论 说明
IHC-P 1/200 - 1/500. Perform heat mediated antigen retrieval with citrate buffer pH 6 before commencing with IHC staining protocol.

靶标

  • 功能May be a receptor protein that acts in collecting-duct and biliary differentiation.
  • 组织特异性Predominantly expressed in fetal and adult kidney. Also present in the adult pancreas, but at much lower levels. Detectable in fetal and adult liver. Rather indistinct signal in fetal brain.
  • 疾病相关Defects in PKHD1 are the cause of polycystic kidney disease autosomal recessive (ARPKD) [MIM:263200]. 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.
  • 序列相似性Contains 2 G8 domains.
    Contains 12 IPT/TIG domains.
    Contains 9 PbH1 repeats.
  • 细胞定位Membrane.
  • Information by UniProt
  • 数据库链接
  • 别名
    • 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
    see all

Anti-PKHD1 antibody 图像

  • Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) analysis of human kidney tissue labelling PKHD1 with ab122160. Staining shows strong cytoplasmic positivity in cells in tubules.

Anti-PKHD1 antibody (ab122160)参考文献

ab122160 has not yet been referenced specifically in any publications.

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