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.
Ten different NPHS2 mutations were found, 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.
Applications:Suitable for use in Western Blot and Immunohistochemistry.
Other applications not tested.
Recommended Dilution:Western Blot: 0.1-0.5ug/mlImmunohistochemistry (paraffin): 0.5-1ug/ml, boiling the paraffin sections in 10mM citrate buffer, pH 6.0, for 20 mins is required for the staining of formalin/paraffin sectionsImmunohistochemistry (frozen): 0.5-1ug/mlOptimal dilutions to be determined by the researcher.
Storage and Stability:Lyophilized and reconstituted products are stable for 12 months after receipt at -20°C.
Reconstitute with sterile ddH2O.
Aliquot to avoid repeated freezing and thawing.
Store at -20°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.
仕様
Size：100ug
Host：rabbit
Source Antibody：human
Grade：Affinity Purified
Purity：Purified by immunoaffinity chromatography.
Form：Supplied as a lyophilized powder from PBS, 5% BSA, 0.05% thimerosal, 0.05% sodium azide. Reconstitute with 200ul sterile dH2O.
Specificity：Recognizes human NPHS2. Species Crossreactivity: mouse and rat.
Isotype：IgG
Calc Applications Abbrev：IHC WB
Calc Crossreactivity：Hu Mo Rt
Immunogen：Synthetic peptide corresponding to aa368-383, KPVEPLNPKKKDSPML, from human NPHS2 at C-terminus, identical to the related mouse sequence, and different from the related rat sequence by 1aa.
