Rabbit Anti-NPHS2/Podocin/FITC Conjugated antibody
|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|
|交叉反应||Human, Mouse, Rat, Dog, Pig, Cow, Horse, Rabbit,|
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|
|免 疫 原||KLH conjugated synthetic peptide derived from human NPHS2/Podocin|
|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.|
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.
Interacts with nephrin/NPHS1 and KIRREL. Interacts directly with CD2AP. Interacts with DDN (By similarity).
Cell membrane; Peripheral membrane protein.
Almost exclusively expressed in the podocytes of fetal and mature kidney glomeruli.
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.
Belongs to the band 7/mec-2 family.
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
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications