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Rabbit Anti-NPHS2/Podocin/Biotin Conjugated antibody (bs-6597R-Bio)
~~~促销,代码KXJ230206-6~~~
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说 明 书: 100ul  
100ul/2980.00元
大包装/询价
产品编号 bs-6597R-Bio
英文名称 Anti-NPHS2/Podocin/Biotin
中文名称 生物素标记的肾小球裂孔膜蛋白PDCN抗体
别    名 nephrosis 2, idiopathic, steroid resistant; NPHS2 gene; PDCN; Podocin; SRN1; PODO_HUMAN.  
规格价格 100ul/2980元 购买        大包装/询价
说 明 书 100ul  
研究领域 细胞生物  信号转导  细胞类型标志物  
抗体来源 Rabbit
克隆类型 Polyclonal
交叉反应 Human, Mouse,  (predicted: Rat, Dog, Pig, Cow, Horse, Rabbit, )
产品应用 WB=1:50-200 ELISA=1:100-1000 IHC-P=1:50-200 IHC-F=1:50-200 IF=1:50-200 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 42kDa
性    状 Lyophilized or Liquid
浓    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human NPHS2/Podocin
亚    型 IgG
纯化方法 affinity purified by Protein A
储 存 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存条件 Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.
产品介绍 background:
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.

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



Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
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