Rabbit NPHS2 Polyclonal Antibody | anti-NPHS2 antibody

Anti-NPHS2 Antibody

Cat. Num. AAA415609

• Gene Names: NPHS2; PDCN; SRN1
• Reactivity: Human, Mouse, Rat. Not yet tested in other species.
• Applications: ELISA, Western Blot
• Purity: Affinity purified
• Synonyms: NPHS2; Polyclonal Antibody; Anti-NPHS2 Antibody; anti-NPHS2 antibody

• Host: Rabbit
• Reactivity: Human, Mouse, Rat. Not yet tested in other species.
• Clonality: Polyclonal
• Isotype: IgG
• Purity/Purification: Affinity purified
• Form/Format: Liquid
• Concentration: 100ug/100ul (varies by lot)
• Sequence Length: 315

• Applicable Applications for anti-NPHS2 antibody: ELISA (EIA), Western Blot (WB)
• Application Notes: Peptide ELISA titer: 1:5,000-1:20,000; Western blot analysis: 1:200-500; Optimal dilutions/concentrations should be determined by the end user.
• Storage Buffer: PBS, pH 7.4 with 0.02% Sodium Azide
• Immunogen: Rabbit polyclonal NPHS2 antibody was raised against a peptide from near the C terminal residues of human NPHS2 protein.
• Preparation and Storage: This product is stable for several weeks at 4 degree C as an undiluted liquid. Dilute only prior to immediate use. For extended storage, aliquot contents and freeze at -20 degree C or below. Avoid cycles of freezing and thawing. Expiration date is one (1) year from date of receipt.

Testing Data

Related Product Information for anti-NPHS2 antibody

This gene encodes the glomerular protein podocin which plays a role in the regulation of glomerular permeability, and acts as a linker between the plasma membrane and the cytoskeleton. Defects in this gene are the cause of autosomal recessive steroid-resistant nephrotic syndrome (SRN). SRN is characterized by onset between three months and five years, resistance to steroid therapy and rapid progression to end-stage renal disease. An alternative splice variant has been described but its full length sequence has not been determined. [provided by RefSeq, Jul 2008]

References

(1) Tonna,S., Wang,Y.Y., Wilson,D., et al.The R229Q mutation in NPHS2 may predispose to proteinuria in thin-basement-membrane ephropathy.Pediatr. Nephrol.23 (12), 2201-2207(2008).

NCBI and Uniprot Product Information

• NCBI GI #: 20809646
• NCBI GeneID: 7827
• Molecular Weight: 34,421 Da
• NCBI Official Full Name: NPHS2 protein
• NCBI Official Synonym Full Names: nephrosis 2, idiopathic, steroid-resistant (podocin)
• NCBI Official Symbol: NPHS2
• NCBI Official Synonym Symbols: PDCN; SRN1
• NCBI Protein Information: podocin
• UniProt Protein Name: Podocin
• Protein Family: Podocin
• UniProt Gene Name: NPHS2
• UniProt Entry Name: PODO_HUMAN

NCBI Description

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]

Uniprot Description

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: protein complex; endoplasmic reticulum; integral to plasma membrane; plasma membrane; intercellular junction; lipid raft

Molecular Function: protein binding

Biological Process: actin cytoskeleton reorganization; excretion

Disease: Nephrotic Syndrome, Type 2