Recombinant Human Nephrin protein (ab114263)

製品の概要

  • 製品名Recombinant Human Nephrin protein
  • タンパク質長Protein fragment

製品の詳細

  • 由来Recombinant
  • 由来Wheat germ
  • アミノ酸配列
    • アクセッション番号O60500
    • 生物種Human
    • 配列GFWALPENLTVVEGASVELRCGVSTPGSAVQWAKDGLLLGPDPRIPGFPR YRLEGDPARGEFHLHIEACDLSDDAEYECQVGRSEMGPEL
    • 分子量36 kDa including tags
    • 領域33 to 122

特性

Our Abpromise guarantee covers the use of ab114263 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

    SDS-PAGE

    Western blot

    ELISA

  • 製品の状態Liquid
  • 備考Protein concentration is above or equal to 0.05mg/ml.
    This protein is best used within three months from the date of receipt.
  • Concentration information loading...

前処理および保存

  • 保存方法および安定性

    Shipped on dry ice. Upon delivery aliquot and store at -80ºC. Avoid freeze / thaw cycles.

    pH: 8.00
    Constituents: 0.3% Glutathione, 0.79% Tris HCl

関連情報

  • 別名
    • CNF
    • Nephrin
    • Nephrosis 1 congenital Finnish type
    • Nephrosis 1, congenital, Finnish type (nephrin)
    • NPHN
    • NPHN_HUMAN
    • NPHS 1
    • Nphs1
    • Renal glomerulus specific cell adhesion receptor
    • Renal glomerulus-specific cell adhesion receptor
    see all
  • 機能Seems to play a role in the development or function of the kidney glomerular filtration barrier. Regulates glomerular vascular permeability. May anchor the podocyte slit diaphragm to the actin cytoskeleton. Plays a role in skeletal muscle formation through regulation of myoblast fusion.
  • 組織特異性Specifically expressed in podocytes of kidney glomeruli.
  • 関連疾患Defects in NPHS1 are the cause of nephrotic syndrome type 1 (NPHS1) [MIM:256300]; also known as Finnish congenital nephrosis (CNF). A renal disease characterized clinically by proteinuria, hypoalbuminemia, hyperlipidemia, and edema. Kidney biopsies show non-specific histologic changes such as focal segmental glomerulosclerosis and diffuse mesangial proliferation. Some affected individuals have an inherited steroid-resistant form and progress to end-stage renal failure.
  • 配列類似性Belongs to the immunoglobulin superfamily.
    Contains 1 fibronectin type-III domain.
    Contains 8 Ig-like C2-type (immunoglobulin-like) domains.
  • 発生段階In 23-week-old embryo found in epithelial podocytes of the periphery of mature and developing glomeruli.
  • 翻訳後修飾Phosphorylated on tyrosine residues.
  • 細胞内局在Cell membrane. Predominantly located at podocyte slit diaphragm between podocyte foot processes. Also associated with podocyte apical plasma membrane.
  • Information by UniProt

Recombinant Human Nephrin protein 画像

  • ab114263 analysed on a 12.5% SDS-PAGE stained with Coomassie Blue.

Recombinant Human Nephrin protein (ab114263) 使用論文

ab114263 has not yet been referenced specifically in any publications.

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