Nephrin Antibody | MyBioSource MBS151503 product information

product summary

company name :

MyBioSource

product type :

antibody

product name :

Nephrin Antibody

catalog :

MBS151503

quantity :

0.1 mg

price :

345 USD

clonality :

polyclonal

host :

rabbit

conjugate :

nonconjugated

reactivity :

human, mouse, rat

application :

western blot, ELISA, immunohistochemistry, immunocytochemistry, enzyme immunoassay

more info or order :

MyBioSource product webpage

product information

catalog number :

MBS151503

products type :

Antibody

products full name :

Nephrin Antibody

products short name :

Nephrin

products name syn :

Nephrin; CNF; NPHN; nephrin; Nephrin; Renal glomerulus-specific cell adhesion receptor; nephrosis 1, congenital, Finnish type (nephrin)

other names :

nephrin; Nephrin; nephrin; renal glomerulus-specific cell adhesion receptor; nephrosis 1, congenital, Finnish type (nephrin); Renal glomerulus-specific cell adhesion receptor

products gene name :

NPHS1

other gene names :

NPHS1; NPHS1; CNF; NPHN; nephrin; NPHN

uniprot entry name :

NPHN_HUMAN

clonality :

Polyclonal

isotype :

IgG

host :

Rabbit

reactivity :

Human, Mouse, Rat

sequence length :

1241

purity :

Nephrin Antibody is affinity chromatography purified via peptide column.

form :

Liquid

concentration :

1 mg/mL

storage stability :

Nephrin antibody can be stored at 4 degree C for three months and -20 degree C, stable for up to one year. As with all antibodies care should be taken to avoid repeated freeze thaw cycles. Antibodies should not be exposed to prolonged high temperatures.

tested application :

ELISA (EIA), Western Blot (WB), Immunohistochemistry (IHC), Immunofluorescence (IF)

app notes :

Nephrin antibody can be used for detection of Nephrin by Western blot at 1 mug/mL. Antibody can also be used for immunohistochemistry starting at 1 mug/mL. For immunofluorescence start at 10 mug/mL.

other info1 :

Conjugate: Unconjugated. Immunogen: Nephrin antibody was raised against a 14 amino acid synthetic peptide from near the carboxy terminus of human Nephrin. Buffer: Nephrin Antibody is supplied in PBS containing 0.02% sodium azide.

products description :

Nephrin Antibody: Nephrin is strongly expressed in renal glomeruli and is a member of the immunoglobulin family of cell adhesion molecules. Mutations in the Nephrin gene result in congenital nephrotic syndrome, an autosomal-recessive disorder characterized by massive proteinuria in utero and nephrosis at birth. Renal glomeruli allow normal kidneys to filter plasma so that it is very pure. Nephrin is expressed in the podocyte slit-diaphragm of the renal glomeruli in a manner that suggests that Nephrin molecules homodimerize in an anti-parallel fashion similar to cadherin interactions in adherens junctions. Thus, Nephrin may constitute the entire extracellular structure of the slit-diaphragm.

ncbi gi num :

4758822

ncbi acc num :

NP_004637

ncbi gb acc num :

NM_004646.3

uniprot acc num :

O60500

ncbi mol weight :

130,546 Da

ncbi pathways :

Cell-Cell Communication Pathway (477132); Nephrin Interactions Pathway (187228); 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: Immunoglobulin superfamily; Cell adhesion; Membrane protein, integral. Chromosomal Location of Human Ortholog: 19q13.1. Cellular Component: protein complex; cell projection; integral to plasma membrane; plasma membrane; lipid raft. Molecular Function: protein domain specific binding; protein binding; myosin binding; spectrin binding; alpha-actinin binding. Biological Process: skeletal muscle development; positive regulation of actin filament polymerization; glomerular basement membrane development; JNK cascade; cell adhesion; excretion; myoblast fusion. Disease: Nephrotic Syndrome, Type 1

size1 :

0.1 mg

price1 :

345 USD

more info or order :

MyBioSource product webpage