製品の概要

  • 製品名Anti-PKHD1 antibody
    PKHD1 一次抗体 製品一覧
  • 製品の詳細
    Rabbit polyclonal to PKHD1
  • アプリケーション適用あり: IHC-Pmore details
  • 種交差性
    交差種: 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.

  • ポジティブ・コントロール
    • Human liver tissue.

製品の特性

関連製品

アプリケーション

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.

アプリケーション Abreviews 特記事項
IHC-P 1/200 - 1/500. Perform heat mediated antigen retrieval with citrate buffer pH 6 before commencing with IHC staining protocol.

ターゲット情報

  • 機能May be a receptor protein that acts in collecting-duct and biliary differentiation.
  • 組織特異性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.
  • 関連疾患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.
  • 配列類似性Contains 2 G8 domains.
    Contains 12 IPT/TIG domains.
    Contains 9 PbH1 repeats.
  • 細胞内局在Membrane.
  • Information by UniProt
  • 参照データベース
  • 別名
    • 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 画像

  • 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) 使用論文

ab122160 has not yet been referenced specifically in any publications.

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