84-1172-33　Podocin (NPHS2) 100ug　P9104-90

特徴

• 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.
• Applications:Suitable for use in Western Blot.
• Other applications not tested.
• Recommended Dilution:Western Blot: 1ug/ml Optimal dilutions to be determined by the researcher.
• Storage and Stability:Lyophilized powder may be stored at -20°C.
• Stable for 12 months at -20°C.
• Reconstitute with sterile dH2O.
• Aliquot to avoid repeated freezing and thawing.
• Store at -20°C.
• Reconstituted product is stable for 12 months 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.