NPHS1
Reactivity: Human
WB, ELISA
Host: Mouse
Monoclonal
3D2
unconjugated
Application Notes
ELISA. Western blot: 0.5 - 1 μg/mL. Immunohistochemistry on paraffin sections. Other applications not tested. Optimal dilutions are dependent on conditions and should be determined by the user.
Restrictions
For Research Use only
Concentration
1.0 mg/mL
Buffer
PBS containing 0.02 % Sodium azide
Preservative
Sodium azide
Precaution of Use
This product contains sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.
Handling Advice
Avoid repeated freezing and thawing.
Storage
4 °C/-20 °C
Storage Comment
Store undiluted at 2-8 °C for one month or (in aliquots) at -20 °C for longer.
Gu, Zhou, Noble, Border, Cheung, Huang: "Targeting reduction of proteinuria in glomerulonephritis: Maximizing the antifibrotic effect of valsartan by protecting podocytes." in: Journal of the renin-angiotensin-aldosterone system : JRAAS, Vol. 15, Issue 2, pp. 177-89, (2015) (PubMed).
Zhang, Gu, Lawrence, Cheung, Huang: "A plasminogen activator inhibitor type 1 mutant retards diabetic nephropathy in db/db mice by protecting podocytes." in: Experimental physiology, Vol. 99, Issue 5, pp. 802-15, (2014) (PubMed).
Target
Nephrin (NPHS1)
(Nephrosis 1, Congenital, Finnish Type (Nephrin) (NPHS1))
Nephrin is strongly expressed in renal glomeruli and is a member of the immunoglobulin family of cell adhesion molecules. Mutations in the Nephrin gene result in congenital nephrotic syndrome, an autosomal-recessive disorder characterized by massive proteinuria in utero and nephrosis at birth. Renal glomeruli allow normal kidneys to filter plasma so that it is very pure. Nephrin is expressed in the podocyte slit-diaphragm of the renal glomeruli in a manner that suggests that Nephrin molecules homodimerize in an anti-parallel fashion similar to cadherin interactions in adherens junctions. Thus, Nephrin may constitute the entire extracellular structure of the slit-diaphragm. Despite its predicted molecular weight, Nephrin often migrates at a lower than expected size in SDS-PAGE.Synonyms: NPHN, NPHS1