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NPHS2 Recombinant Rabbit mAb (bsm-54280R)  
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產品編號 bsm-54280R
英文名稱 NPHS2 Recombinant Rabbit mAb
中文名稱 腎小球裂孔膜蛋白PDCN重組兔單抗
別    名 nephrosis 2, idiopathic, steroid resistant; NPHS2 gene; PDCN; Podocin; SRN1; PODO_HUMAN.  
研究領域 細胞生物  信號轉導  細胞類型標志物  
抗體來源 Rabbit
克隆類型 Recombinant
克 隆 號 1A5
交叉反應 Human,Mouse,Rat
產品應用 WB=1:500-2000,IHC-P=1:50-200,IHC-F=1:400-800,IF=1:100-500
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
理論分子量 42 kDa
檢測分子量
性    狀 Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human NPHS2 
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
產品介紹 Podocin is an important member of a group of proteins shown to be associated with the slit diaphragm. Podocin belongs to the band 7 stomatin family of lipid raft-associated proteins. It is a hairpin like integral membrane protein with intracellular N and C termini. Podocin is located at the insertion site of the slit membrane, and is thought to act as a scaffold protein required to maintain or regulate the structural integrity of the slit diaphragm. It plays a role in the regulation of glomerular permeability, acting probably as a linker between the plasma membrane and the cytoskeleton.

Subunit:
Interacts with nephrin/NPHS1 and KIRREL. Interacts directly with CD2AP. Interacts with DDN (By similarity).

Subcellular Location:
Cell membrane; Peripheral membrane protein.

Tissue Specificity:
Almost exclusively expressed in the podocytes of fetal and mature kidney glomeruli.

DISEASE:
Defects in NPHS2 are the cause of nephrotic syndrome type 2 (NPHS2) [MIM:600995]. It is a renal disorder characterized clinically by childhood onset of proteinuria, hypoalbuminemia, hyperlipidemia, and edema. Kidney biopsies show non-specific histologic changes such as focal segmental glomerulosclerosis and diffuse mesangial proliferation. The disorder is resistant to steroid treatment and progresses to end-stage renal failure in the first or second decades. Some patients show later onset of the disorder.

Similarity:
Belongs to the band 7/mec-2 family.

SWISS:
Q9NP85

Gene ID:
7827

Database links:

Entrez Gene: 7827 Human

Entrez Gene: 170484 Mouse

Entrez Gene: 170672 Rat

Omim: 604766 Human

SwissProt: Q9NP85 Human

SwissProt: Q91X05 Mouse

SwissProt: Q8K4G9 Rat

Unigene: 412710 Human

Unigene: 289099 Mouse

Unigene: 86433 Rat



產品圖片
Sample: Lane 1: rat kidney tissue lysate Lane 2: human kidney tissue lysate Primary: Anti-NPHS2 (bsm-54280R) at 1:500 dilution Secondary: Goat Anti-Rabbit IgG - HRP at 1:5000 dilution Predicted band size: 42 kD Observed band size: 42 kD
Paraformaldehyde-fixed, paraffin embedded (mouse kidney); Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15min; Block endogenous peroxidase by 3% hydrogen peroxide for 20 minutes; Blocking buffer (normal goat serum) at 37°C for 30min; Antibody incubation with (NPHS2) Monoclonal Antibody, Unconjugated (bsm-54280R) at 1:50 overnight at 4°C, followed by operating according to SP Kit(Rabbit) (sp-0023) instructionsand DAB staining.
Paraformaldehyde-fixed, paraffin embedded (rat kidney); Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15min; Block endogenous peroxidase by 3% hydrogen peroxide for 20 minutes; Blocking buffer (normal goat serum) at 37°C for 30min; Antibody incubation with (NPHS2) Monoclonal Antibody, Unconjugated (bsm-54280R) at 1:50 overnight at 4°C, followed by operating according to SP Kit(Rabbit) (sp-0023) instructionsand DAB staining.
Paraformaldehyde-fixed, paraffin embedded (human kidney); Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15min; Block endogenous peroxidase by 3% hydrogen peroxide for 20 minutes; Blocking buffer (normal goat serum) at 37°C for 30min; Antibody incubation with (NPHS2) Monoclonal Antibody, Unconjugated (bsm-54280R) at 1:50 overnight at 4°C, followed by operating according to SP Kit(Rabbit) (sp-0023) instructionsand DAB staining.
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