The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
1/10000 - 1/50000. Detects a band of approximately 138, 180 kDa (predicted molecular weight: 138 kDa).
FunctionSeems to play a role in the development or function of the kidney glomerular filtration barrier. Regulates glomerular vascular permeability. May anchor the podocyte slit diaphragm to the actin cytoskeleton. Plays a role in skeletal muscle formation through regulation of myoblast fusion.
Tissue specificitySpecifically expressed in podocytes of kidney glomeruli.
Involvement in diseaseDefects in NPHS1 are the cause of nephrotic syndrome type 1 (NPHS1) [MIM:256300]; also known as Finnish congenital nephrosis (CNF). A renal disease characterized clinically by proteinuria, hypoalbuminemia, hyperlipidemia, and edema. Kidney biopsies show non-specific histologic changes such as focal segmental glomerulosclerosis and diffuse mesangial proliferation. Some affected individuals have an inherited steroid-resistant form and progress to end-stage renal failure.
Sequence similaritiesBelongs to the immunoglobulin superfamily. Contains 1 fibronectin type-III domain. Contains 8 Ig-like C2-type (immunoglobulin-like) domains.
Developmental stageIn 23-week-old embryo found in epithelial podocytes of the periphery of mature and developing glomeruli.
Post-translational modificationsPhosphorylated on tyrosine residues.
Cellular localizationCell membrane. Predominantly located at podocyte slit diaphragm between podocyte foot processes. Also associated with podocyte apical plasma membrane.
Western blot - Nephrin (phospho Y1176 + Y1193) antibody [EPTPG1] (ab80299)
All lanes : Anti-Nephrin (phospho Y1176 + Y1193) antibody [EPTPG1] (ab80299) at 1/50000 dilution
Lane 1 : HEK293T cells transfected with wild type CD 16/7 Nephrin Lane 2 : HEK293T cells transfected with active tyrosine kinase (vSrc) Lane 3 : HEK293T cells transfected with Nephrin mutated at Tyr1176 and vSrc Lane 4 : HEK293T cells transfected with Nephrin mutated at Tyr 1193 and vSrc using Phospho Nephrin (pY1176/1193)
Secondary HRP labelled goat anti-rabbit at 1/2000 dilution