Nephrosis 2, Idiopathic, Steroid-Resistant (Podocin) (NPHS2) antibody

Antigen: Nephrosis 2, Idiopathic, Steroid-Resistant (Podocin) (NPHS2)

Synonyms: PDCN, SRN1, AI790225, podocin, MGC112589, nphs2l, si:dkey-171o17.6

Clonality: Polyclonal

Host: Please enquire

Reactivity: Mouse (Murine), Rat (Rattus)

Conjugate: Un-conjugated

Application: Western Blotting (WB)

Catalog no.: ABIN966872

Additional Information

Alternative name: Podocin

Immunogen: A synthetic peptide corresponding to a sequence at the middle region of human Podocin, different from the mouse sequence by one amino acid.

Reconstitution: 0.2ml of distilled water will yield a concentration of 500µg/ml.

Description: 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.1

Application Details

Buffer: Each vial contains 5mg BSA, 0.9mg NaCl, 0.2mg Na2HPO4

Preservative: 0.05mg Thiomersal, 0.05mg NaN3.

Storage: At -20°C for one year. After reconstitution, at 4°C for one month.

Research Area: Cell/Tissue Markers, Signaling

Restrictions: For Research Use only

Publications

Product: Boute, Gribouval, Roselli et al.: "NPHS2, encoding the glomerular protein podocin, is mutated in autosomal recessive steroid-resistant nephrotic syndrome." in: Nature genetics, Vol. 24, Issue 4, pp. 349-54, 2000 (PubMed).