NPHS1 | Nephrin Rabbit pAb(bs-10233R)-50ul/100ul/200ul产品图
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NPHS1 | Nephrin Rabbit pAb(bs-

10233R)-50ul/100ul/200ul
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  • ¥1180 - 2800
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  • bs-10233R
  • 2026年10月17日
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      50ul/100ul/200ul

    规格:50ul产品价格:¥1180.0
    规格:100ul产品价格:¥1980.0
    规格:200ul产品价格:¥2800.0
    产品编号bs-10233R
    英文名称Nephrin Rabbit pAb
    中文名称肾小球细胞粘附分子受体抗体
    英文别名CNF; NPHN; Nephrosis 1 congenital Finnish type; NPHS 1; NPHS1; Renal glomerulus specific cell adhesion receptor; Renal glomerulus-specific cell adhesion receptor; NPHN_HUMAN .
    产品应用WB=1:500-2000, IHC-P=1:100-500, IHC-F=1:100-500, IF=1:100-500, Flow-Cyt=1μg/Test

    Not yet tested in other applications.
    Optimal working dilutions must be determined by the end user.

    交叉反应Human, Mouse, Rat, Dog (Rabbit)
    抗体来源Rabbit
    免疫原KLH conjugated synthetic peptide derived from human Nephrin
    亚型IgG
    性状Liquid
    纯化方法affinity purified by Protein A
    克隆类型Polyclonal
    理论分子量138 kDa
    浓度1mg/ml
    储存液0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
    研究领域

    Signal Transduction > Cytoskeleton / ECM > Cell Adhesion > Cell Adhesion Molecules > Endothelial

    亚基Interacts with CD2AP (via C-terminal domain). Interacts with MAGI1 (via PDZ 2 and 3 domains) forming a tripartite complex with IGSF5/JAM4. Interacts with DDN; the interaction is direct. Self-associates (via the Ig-like domains). Also interacts (via the Ig-like domains) with KIRREL/NEPH1 and KIRREL2; the interaction with KIRREL is dependent on KIRREL glycosylation. Forms a complex with ACTN4, CASK, IQGAP1, MAGI2, SPTAN1 and SPTBN1 (By similarity). Interacts with NPHS2.
    亚细胞定位Cell membrane; Single-pass type I membrane protein (Potential). Note=Predominantly located at podocyte slit diaphragm between podocyte foot processes. Also associated with podocyte apical plasma membrane.
    组织特异性Specifically expressed in podocytes of kidney glomeruli. Expressed in kidney glomeruli. In the embryo,expressed in the mesonephric kidney at E11 with strong expression in cranial tubules with podocyte-like structures. Expression is observed in the podocytes of the developing kidney from E13. High expression is also detected in the developing cerebellum, hindbrain, spinal cord, retina and hypothalamus. Expressed in skeletal muscle during myoblast fusion such as in the adult following acute injury and in the embryo but not detected in uninjured adult skeletal muscle. Isoform 1 and isoform 2 are expressed in the newborn brain and developing cerebellum. Isoform 1 is the predominant isoform in adult kidney.
    翻译后修饰Phosphorylated at Tyr-1193 by FYN, leading to the recruitment and activation of phospholipase C-gamma-1/PLCG1._x000D_ 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.
    相似性Belongs to the immunoglobulin superfamily.
    Contains 1 fibronectin type-III domain.
    Contains 8 Ig-like C2-type (immunoglobulin-like) domains.
    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.
    保存条件Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles.
    注意事项This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
    背景资料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]

     

    应用推荐稀释比例
    {WB}{1:500-2000}
    {IHC-P}{1:100-500}
    {IHC-F}{1:100-500}
    {IF}{1:100-500}
    {Flow-Cyt}{1μg/Test}

     

    产品细节图片1
    Sample:
    _x000D_ MDCK(Dog) Cell Lysate at 40 ug
    _x000D_ Primary: Anti-Nephrin (bs-10233R) at 1/500 dilution
    _x000D_ Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution
    _x000D_ Predicted band size: 138 kD
    _x000D_ Observed band size: 138 kD
    产品细节图片2
    Sample:
    _x000D_ Liver (Mouse) Lysate at 40 ug
    _x000D_ Primary: Anti-Nephrin (bs-10233R) at 1/300 dilution
    _x000D_ Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution
    _x000D_ Predicted band size: 138 kD
    _x000D_ Observed band size: 138 kD
    _x000D_
    产品细节图片3
    Blank control:293T.
    _x000D_ Primary Antibody (green line): Rabbit Anti-Nephrin antibody (bs-10233R)
    _x000D_ Dilution: 1ug/Test;
    _x000D_ Secondary Antibody : Goat anti-rabbit IgG-FITC
    _x000D_ Dilution: 0.5ug/Test.
    _x000D_ Protocol
    _x000D_ The cells wereincubated in 5%BSA to block non-specific protein-protein interactions for 30 min at room temperature .Cells stained with Primary Antibody for 30 min at room temperature. The secondary antibody used for 40 min at room temperature. Acquisition of 20,000 events was performed._x000D_
    产品细节图片4
    Tissue/cell: mouse kidney tissue; 4% P‌‌araformaldehyde-fixed and paraffin-embedded;
    _x000D_ Antigen retrieval: citrate buffer ( 0.01M, pH 6.0 ), Boiling bathing for 15min; Block endogenous peroxidase by 3% Hydrogen peroxide for 30min; Blocking buffer (normal goat serum,C-0005) at 37℃ for 20 min;
    _x000D_ Incubation: Anti-Nephrin Polyclonal Antibody, Unconjugated(bs-10233R) 1:200, overnight at 4°C, followed by conjugation to the secondary antibody(SP-0023) and DAB(C-0010) staining
    _x000D_
    产品细节图片5
    the cells(293T) were fixed with 2% P‌‌araformaldehyde (10 min).
    _x000D_ Isotype Control Antibody: Rabbit IgG(orange) ; _x000D_ Secondary Antibody: Goat anti-rabbit IgG-PE(white blue), _x000D_ Dilution: 1:200 in 1 X PBS containing 0.5% BSA ; _x000D_ Primary Antibody Dilution: 1μg in 100 μL1X PBS containing 0.5% BSA(green).
    产品细节图片6
    Blank control: RSC96(blue), the cells were fixed with 2% P‌‌araformaldehyde (10 min) and then permeabilized with ice-cold 90% methanol for 30 min on ice.
    _x000D_ Isotype Control Antibody: Rabbit IgG(orange) ; _x000D_ Secondary Antibody: Goat anti-rabbit IgG-PE(white blue), _x000D_ Dilution: 1:200 in 1 X PBS containing 0.5% BSA ; _x000D_ Primary Antibody Dilution: 1μg in 100 μL1X PBS containing 0.5% BSA(green)._x000D_

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    NPHS1 | Nephrin Rabbit pAb(bs-10233R)-50ul/100ul/200ul
    ¥1180 - 2800