Recombinant人Nephrin protein (ab114263)

概述

  • 产品名称
    Recombinant人Nephrin protein
  • 蛋白长度
    Protein fragment

描述

  • 性质
    Recombinant
  • 来源
    Wheat germ
  • 氨基酸序列
    • Accession
    • 种属
      Human
    • 序列
      GFWALPENLTVVEGASVELRCGVSTPGSAVQWAKDGLLLGPDPRIPGFPR YRLEGDPARGEFHLHIEACDLSDDAEYECQVGRSEMGPEL
    • 分子量
      36 kDa including tags
    • 氨基酸
      33 to 122

技术指标

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.

  • 应用

    SDS-PAGE

    Western blot

    ELISA

  • 形式
    Liquid
  • 补充说明
    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...

制备和贮存

  • 稳定性和存储

    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

常规信息

  • 别名
    • 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
  • 功能
    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.
  • 组织特异性
    Specifically expressed in podocytes of kidney glomeruli.
  • 疾病相关
    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.
  • 序列相似性
    Belongs to the immunoglobulin superfamily.
    Contains 1 fibronectin type-III domain.
    Contains 8 Ig-like C2-type (immunoglobulin-like) domains.
  • 发展阶段
    In 23-week-old embryo found in epithelial podocytes of the periphery of mature and developing glomeruli.
  • 翻译后修饰
    Phosphorylated on tyrosine residues.
  • 细胞定位
    Cell membrane. Predominantly located at podocyte slit diaphragm between podocyte foot processes. Also associated with podocyte apical plasma membrane.
  • Information by UniProt

图片

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

文献

ab114263 has not yet been referenced specifically in any publications.

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