Nephrosis 1, Congenital, Finnish Type (Nephrin) (NPHS1) (C-Term) antibody

Antigen: Nephrosis 1, Congenital, Finnish Type (Nephrin) (NPHS1)

Synonyms: CNF, NPHN, nephrin, NephrinB, NPHS1, nphs1

Epitope: C-Term

Clonality: Polyclonal

Host: Rabbit

Reactivity: Human

Application: Immunohistochemistry (Frozen Sections) (IHC (fro)), Western Blotting (WB), Immunoprecipitation (IP)

Catalog no.: ABIN195447

Additional Information

Alternative name: Nephrin (CT)

Immunogen: Synthetic peptide of the C-terminal part of Human Nephrin

Format: Lyophilized

Isotype: IgG  (Matching secondary antibodies)

Description: Nephrin has been identified as a disease causing molecule responsible for congenital nephrotic syndrome of Finnish type, in which the proteinuria starts in utero or immediately after birth (ref. 1). It is an immunoglobulin-like transmembrane adhesion protein having eight Ig motifs. Nephrin forms a structural backbone of the slit diaphragm through a head-to-head, trans-homo-interaction at the N-terminal ectodomains. In addition to the structural function, nephrin serves as a signaling molecule that mediate an adaptive morphological regulation through the remodeling of actin cytoskeletons, the C-terminal cytoplasmic domain is tyrosine-phosphorylated by Src family kinase (Fyn) and the phosphorylated nephrin associates with an SH2 adaptor protein (Nck), thereby allowing the sable anchorage of the slit membrane complex to the actin cytoskeleton in the foot processes. Under proteinuric conditions, nephrin is down-regulated and is apically dislocated (ref. 2).

Specificity: Cross-reacts with rat

Application Details

Application Notes: For immunohistochemistry in a frozen section, the starting concentration is 5 ?g/mL (mainly can be used for study of cell surface). For western blotting, the starting concentration is about 0.05 - 0.5 ?g /mL. For immuno-precipitation, the staring concentration is about 1 - 2 ?g /mL.

Purification: Purified with antigen

Buffer: Lyophilized product from 1% BSA in PBS containing 0.05% NaN3

Storage: 1.0 mL deionized water will be added to the product (the conc. comes up 100 ug/ml). Lyophilized product, 5 years at 2 – 8 °C. Solution, 2 years at –20 °C

Research Area: Cell/Tissue Markers, Extracellular Matrix, Signaling

Restrictions: For Research Use only

Images

Rat renal glomerular (FITC)

Human renal glomerular (DAB)

anti-Nephrosis 1, Congenital, Finnish Type (Nephrin) (NPHS1) (C-Term) antibody (Image 3)