Rabbit anti-Human, Rat Podocin Polyclonal Antibody | anti-NPHS2 antibody

Podocin (NPHS2)

Cat. Num. AAA626189

• Gene Names: NPHS2; PDCN; SRN1
• Reactivity: Human, Rat
• Applications: Western Blot
• Purity: Affinity Purified; Purified by immunoaffinity chromatography.
• Synonyms: Podocin; Polyclonal Antibody; Podocin (NPHS2); Anti -Podocin (NPHS2); anti-NPHS2 antibody

• Host: Rabbit
• Reactivity: Human, Rat
• Clonality: Polyclonal
• Isotype: IgG
• Specificity: Recognizes human podocin. Species Crossreactivity
• Purity/Purification: Affinity Purified; Purified by immunoaffinity chromatography.
• Form/Format: Supplied as a lyophilized powder from PBS, 5% BSA, 0.05% Thimerosal, 0.05% sodium azide. Reconstitute with 200ul sterile ddH2O.

• Applicable Applications for anti-NPHS2 antibody: Western Blot (WB)
• Application Notes: Suitable for use in Western Blot.; Dilution: Western Blot: 1ug/ml
• Immunogen: Synthetic peptide corresponding to a sequence at the middle region of human Podocin.
• Preparation and Storage: Lyophilized powder may be stored at -20 degree C. Stable for 12 months at -20 degree C. Reconstitute with sterile dH2O. Aliquot to avoid repeated freezing and thawing. Store at -20 degree C. Reconstituted product is stable for 12 months at -20 degree C. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap. Further dilutions can be made in assay buffer.

Related Product Information for anti-NPHS2 antibody

Podocin (PDCN) is a protein which lines the podocytes and assists in maintaining the barrier at the glomerular basement membrane. NPHS2 is a causative gene for Familial idiopathic nephrotic syndromes, which represents a heterogeneous group of kidney disorders, and include autosomal recessive steroid-resistant nephrotic syndrome, which is characterized by early childhood onset of proteinuria, rapid progression to end-stage renal disease and focal segmental glomerulosclerosis. By positional cloning, NPHS2 was mapped to 1q25-31. It is almost exclusively expressed in the podocytes of fetal and mature kidney glomeruli, and encodes a new integral membrane protein, podocin, belonging to the stomatin protein family. Boute et al. (2000) found ten different NPHS2 mutations, comprising nonsense, frameshift and missense mutations, to segregate with the disease, demonstrating a crucial role for podocin in the function of the glomerular filtration barrier.

Product Categories/Family for anti-NPHS2 antibody

Antibodies; Abs to Cell Markers

NCBI and Uniprot Product Information

• NCBI GI #: 7657615
• NCBI GeneID: 7827
• NCBI Accession #: NP_055440.1
• NCBI GenBank Nucleotide #: NM_014625.2
• UniProt Accession #: Q9NP85; Q8N6Q5; B1AM32; B1AM33
• Molecular Weight: 42,201 Da
• NCBI Official Full Name: podocin
• 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; OTTHUMP00000033186; OTTHUMP00000033187
• UniProt Protein Name: Podocin
• Protein Family: Podocin
• UniProt Gene Name: NPHS2
• UniProt Entry Name: PODO_HUMAN

NCBI Description

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]

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; integral to plasma membrane; endoplasmic reticulum; plasma membrane; intercellular junction; lipid raft

Molecular Function: protein binding

Biological Process: actin cytoskeleton reorganization; excretion

Disease: Nephrotic Syndrome, Type 2