产品名称
Nephrin Rabbit Polyclonal Antibody
别名
NPHS1; NPHN; Nephrin; Renal glomerulus-specific cell adhesion receptor
存储缓冲液
Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% New type preservative N.
Human Gene Link
http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&term=4868
Human Swissprot No.
O60500
Human Swissprot Link
http://www.uniprot.org/uniprotkb/O60500/entry
Mouse Gene Link
http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&term=54631
Mouse Swissprot No.
Q9QZS7
Mouse Swissprot Link
http://www.uniprot.org/uniprot/Q9QZS7
Rat Gene Link
http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&term=64563
Rat Swissprot Link
http://www.uniprot.org/uniprot/Q9R044
免疫原
The antiserum was produced against synthesized peptide derived from human Nephrin. AA range:843-892
特异性
Nephrin Polyclonal Antibody detects endogenous levels of Nephrin protein.
稀释度
WB 1:500-2000 IHC 1:100 - 1:300. ELISA: 1:40000. IF 1:50-200
宿主
Polyclonal, Rabbit,IgG
背景介绍
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],
组织表达
Specifically expressed in podocytes of kidney glomeruli.
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. .
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.,subunit:Interacts with podocin/NPHS2 and KIRREL. Interacts with CD2AP C-terminal domain (By similarity). Interacts with MAGI1 PDZ 2 and 3 domains forming a tripartite complex with IGSF5/JAM4 (By similarity). Interacts with DDN; the interaction is direct.,tissue specificity:Specifically expressed in podocytes of kidney glomeruli.,
纯化
The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.