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).
機能Seems 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.
組織特異性Specifically expressed in podocytes of kidney glomeruli.
関連疾患Defects 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.
配列類似性Belongs to the immunoglobulin superfamily. Contains 1 fibronectin type-III domain. Contains 8 Ig-like C2-type (immunoglobulin-like) domains.
発生段階In 23-week-old embryo found in epithelial podocytes of the periphery of mature and developing glomeruli.
翻訳後修飾Phosphorylated on tyrosine residues.
細胞内局在Cell 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