免疫原
The antiserum was produced against synthesized peptide derived from human Nephrin. AA range:843-892
特异性
Nephrin Monoclonal Antibody detects endogenous levels of Nephrin protein.
组成（Formulation）
Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
纯化工艺（Immunogen）
The antibody was affinity-purified from mouse antiserum by affinity-chromatography using epitope-specific immunogen.
背景（Background）
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],
功能
developmental stage:In 23-week-old embryo found in epithelial podocytes of the periphery of mature and developing glomeruli.,disease:Defects in NPHS1 are the cause of congenital nephrotic syndrome of the Finnish type (NPHS1 or CNF) [MIM:256300]. CNF is an autosomal recessive disorder characterized by massive proteinuria in utero and nephrosis at birth.,function:Seems to play a role in the development or function of the kidney glomerular filtration barrier. May anchor the podocyte slit diaphragm to the actin cytoskeleton.,PTM:Phosphorylated on tyrosine residues.,similarity:Belongs to the immunoglobulin superfamily.,similarity:Contains 1 fibronectin type-III domain.,similarity:Contains 8 Ig-like C2-type (immunoglobulin-like) domains.,subcellular location:Predominantly located at podocyte slit diaphragm between podocyte foot processes. Also associated with podocyte apical plasma membrane.,s
其他名称
NPHS1; NPHN; Nephrin; Renal glomerulus-specific cell adhesion receptor
细胞定位
Cell membrane ; Single-pass type I membrane protein . Predominantly located at podocyte slit diaphragm between podocyte foot processes. Also associated with podocyte apical plasma membrane. .
组织表达
Specifically expressed in podocytes of kidney glomeruli.
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