catalog number :
MBS7000356
products full name :
Rabbit anti-human Nephrin polyclonal Antibody
products short name :
[Nephrin]
products name syn :
[Nephrin (Renal glomerulus-specific cell adhesion receptor), NPHS1, NPHN]
other names :
[nephrin; Nephrin; nephrin; NPHS1 nephrin; Renal glomerulus-specific cell adhesion receptor]
products gene name syn :
[NPHS1]
other gene names :
[NPHS1; NPHS1; CNF; NPHN; nephrin; NPHN]
uniprot entry name :
NPHN_HUMAN
purity :
>95%, Protein G purified
storage stability :
Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
tested application :
ELISA (EIA), Western Blot (WB), Immunohistochemistry (IHC), Immunofluorescence (IF)
app notes :
Recommended dilution:. WB:1:500-1:5000. IHC:1:200-1:500. IF:1:50-1:200
image1 heading :
Immunohistochemistry (IHC)
image2 heading :
Western Blot (WB)
image3 heading :
Immunofluorescence (IF)
other info1 :
Immunogen: Recombinant Human Nephrin protein (23-257AA). Conjugate: Non-conjugated
other info2 :
Buffer: Preservative: 0.03% Proclin 300. Constituents: 50% Glycerol, 0.01M PBS, PH 7.4. Santa Cruz Alternative: Potential replacement for Santa Cruz Biotechnology antibody catalog# sc-19000 / sc-32529 / sc-32530 / sc-32532 / sc-28192. Species: Human. Research Area: Signal Transduction. Target Names: NPHS1
products description :
Seems to play a role in the development or function of the kidney glomerular filtration barrier. Regulates glomerular vascular permeability. May anchor the podocyte slit diaphragm to the actin cytoskeleton. Plays a role in skeletal muscle formation through regulation of myoblast fusion.
ncbi acc num :
NP_004637.1
ncbi gb acc num :
NM_004646.3
ncbi pathways :
Cell-Cell Communication Pathway (1270231); Nephrin Interactions Pathway (1270243); Nephrin/Neph1 Signaling In The Kidney Podocyte Pathway (138072)
ncbi summary :
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]
uniprot summary :
NPHS1: Seems to play a role in the development or function of the kidney glomerular filtration barrier. Regulates glomerular vascular permeability. May anchor the podocyte slit diaphragm to the actin cytoskeleton. Plays a role in skeletal muscle formation through regulation of myoblast fusion. Interacts with CD2AP (via C-terminal domain). Interacts with MAGI1 (via PDZ 2 and 3 domains) forming a tripartite complex with IGSF5/JAM4. Interacts with DDN; the interaction is direct. Self-associates (via the Ig-like domains). Also interacts (via the Ig-like domains) with KIRREL/NEPH1 and KIRREL2; the interaction with KIRREL is dependent on KIRREL glycosylation. Forms a complex with ACTN4, CASK, IQGAP1, MAGI2, SPTAN1 and SPTBN1. Interacts with NPHS2. Specifically expressed in podocytes of kidney glomeruli. Belongs to the immunoglobulin superfamily. 2 isoforms of the human protein are produced by alternative splicing. Protein type: Membrane protein, integral; Immunoglobulin superfamily; Cell adhesion. Chromosomal Location of Human Ortholog: 19q13.1. Cellular Component: cell projection; integral to plasma membrane; intracellular; plasma membrane. Molecular Function: myosin binding; protein binding. Biological Process: cell adhesion; excretion; glomerular basement membrane development; JNK cascade; myoblast fusion; positive regulation of actin filament polymerization; skeletal muscle development. Disease: Nephrotic Syndrome, Type 1