antibody

NPHS2 Rabbit Polyclonal

MBS7602052

0.1 mg

265 USD

polyclonal

rabbit

nonconjugated

human, mouse, rat

western blot, ELISA, immunocytochemistry, enzyme immunoassay

Antibody

NPHS2 Rabbit Polyclonal

[NPHS2]

[NPHS2 protein; Podocin; podocin; NPHS2 podocin]

[NPHS2]

[NPHS2; NPHS2; PDCN; SRN1]

PODO_HUMAN

Polyclonal

IgG

Rabbit

Human, Mouse, Rat

315

Human, Mouse, Rat; other species are not tested. Please decide the specificity by homology

>=95% as determined by SDS-PAGE. Immunogen Affinity Purified

Liquid

PBS with 0.02% sodium azide and 50% glycerol pH 7.3, -20 degree C for 24 months (Avoid repeated freeze / thaw cycles.)

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

WB: 1:200-1:1000; IHC: 1:200-1:1000; IF: 1:20-1:200

Immunohistochemistry

SDS-PAGE

Immunogen: Nephrosis 2, idiopathic, steroid-resistant (podocin)

Plays a role in the regulation of glomerular permeability, acting probably as a linker between the plasma membrane and the cytoskeleton.

20809646

AAH29141.1

34kd

Nephrin/Neph1 Signaling In The Kidney Podocyte Pathway (138072)

This gene encodes a protein that plays a role in the regulation of glomerular permeability. Mutations in this gene cause steroid-resistant nephrotic syndrome. Alternative splicing results in multiple transcript variants. [provided by RefSeq, Jul 2014]

NPHS2: Plays a role in the regulation of glomerular permeability, acting probably as a linker between the plasma membrane and the cytoskeleton. Defects in NPHS2 are the cause of nephrotic syndrome type 2 (NPHS2). 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. 2 isoforms of the human protein are produced by alternative splicing. Protein type: Membrane protein, integral. Chromosomal Location of Human Ortholog: 1q25.2. Cellular Component: endoplasmic reticulum; integral to plasma membrane; intercellular junction; lipid raft; plasma membrane; protein complex. Molecular Function: protein binding. Biological Process: actin cytoskeleton reorganization; excretion. Disease: Nephrotic Syndrome, Type 2

0.1 mg

265 USD