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    phospho-Nephrin (Tyr1217) Rabbit pAb (bs-19199R)  
    訂購熱線(xiàn):400-901-9800
    訂購郵箱:sales@bioss.com.cn
    訂購QQ:  400-901-9800
    技術(shù)支持:techsupport@bioss.com.cn
    50ul/1180.00元
    100ul/1980.00元
    200ul/2800.00元
    大包裝/詢(xún)價(jià)
    產(chǎn)品編號 bs-19199R
    英文名稱(chēng) phospho-Nephrin (Tyr1217) Rabbit pAb
    中文名稱(chēng) 磷酸化腎小球細胞粘附分子受體抗體
    別    名 Nephrin(phospho Y1217): p-Nephrin(phospho Y1217); 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.  
    產(chǎn)品類(lèi)型 磷酸化抗體 
    研究領(lǐng)域 細胞生物  信號轉導  細胞粘附分子  細胞骨架  
    抗體來(lái)源 Rabbit
    克隆類(lèi)型 Polyclonal
    克 隆 號
    交叉反應 Human (predicted: Mouse,Rat)
    產(chǎn)品應用 WB=1:500-2000,ELISA=1:5000-10000
    not yet tested in other applications.
    optimal dilutions/concentrations should be determined by the end user.
    理論分子量 136 kDa
    檢測分子量
    細胞定位 細胞膜 
    性    狀 Liquid
    濃    度 1mg/ml
    免 疫 原 KLH conjugated synthesised phosphopeptide derived from human Nephrin around the phosphorylation site of Tyr1217: GI(p-Y)DQ 
    亞    型 IgG
    純化方法 affinity purified by Protein A
    緩 沖 液 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
    保存條件 Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles.
    注意事項 This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
    PubMed PubMed
    產(chǎn)品介紹 This gene encodes a member of the immunoglobulin family of cell adhesion molecules that functions in the glomerular filtration barrier in the kidney. The gene is primarily expressed in renal tissues, and the protein is a type-1 transmembrane protein found at the slit diaphragm of glomerular podocytes. The slit diaphragm is thought to function as an ultrafilter to exclude albumin and other plasma macromolecules in the formation of urine. Mutations in this gene result in Finnish-type congenital nephrosis 1, characterized by severe proteinuria and loss of the slit diaphragm and foot processes.[provided by RefSeq, Oct 2009]

    Function:
    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.

    Subcellular Location:
    Cell membrane. Predominantly located at podocyte slit diaphragm between podocyte foot processes. Also associated with podocyte apical plasma membrane.

    Tissue Specificity:
    Specifically expressed in podocytes of kidney glomeruli.

    Post-translational modifications:
    Phosphorylated on tyrosine residues.

    DISEASE:
    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.

    Similarity:
    Belongs to the immunoglobulin superfamily.
    Contains 1 fibronectin type-III domain.
    Contains 8 Ig-like C2-type (immunoglobulin-like) domains.

    SWISS:
    O60500

    Gene ID:
    4868

    Database links:

    Entrez Gene: 4868 Human

    Entrez Gene: 54631 Mouse

    Omim: 602716 Human

    SwissProt: O60500 Human

    SwissProt: Q9QZS7 Mouse

    Unigene: 122186 Human

    Unigene: 437830 Mouse



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