Anti-Nephrin Antibody, clone 3B6.1

产品说明

一般描述

Nephrin (UniProt O60500; also known as Renal glomerulus-specific cell adhesion receptor) is encoded by the NPHS1 (also known as NPHN) gene (Gene ID 4868) in human. The transmembrane protein nephrin is an essential component of the podocyte slit diaphragm, the main functional unit of the glomerulus. The renal filtration barrier consists of fenestrated endothelial cells, the glomerular basement membrane (GBM), and the podocytes of epithelial cells. The interdigitating podocyte foot processes are bridged by a 40 nm wide extracellular structure known as the slit diaphragm. Together with the capillary endothelium and GBM, podocytes comprise the glomerular filtration barrier. The GBM functions as a coarse prefilter for large plasma proteins, whereas the slit diaphragm serves as an isoporous ultrafilter with pores smaller than albumin. Nephrin gene mutations are linked to the autosomal recessive disorder congenital nephrotic syndrome of the Finnish type (NPHS1) characterized by massive proteinuria already in utero and nephrosis soon after birth. Both nephrin and the slit diaphragm are reported to be absent in NPHS1 cases. Nephrin is produced with a signal peptide (a.a. 1-22), the removal of which yields the mature protein with a large extracellular region (a.a. 23-1055), followed by a transmembrane segment (a.a. 1056-1076) and a cytoplasmic tail (a.a. 1077-1241). The extracellular region contains eight C-type Ig-like domains (a.a. 27-939), and a Fibronectin type III domain (a.a. 943-1038), while the cytoplasmic tail contains the NPHS2-binding region (a.a. 1160-1241).

特异性

Clone 3B6.1 recognizes an extracellular epitope in the fifth C2-type Ig-like domain that is present in both spliced isoforms of human nephrin reported by UniProt (O60500).

免疫原

Epitope: C2-type Ig-like domain 5.
GST-tagged recombinant fragment corresponding to the fifth C2-type Ig-like domain of human Nephrin.

应用

Research Category
Cell Structure
Research Sub Category
Renal Physiology
Anti-Nephrin Antibody, clone 3B6.1 is an antibody against Nephrin for use in Western Blotting.

质量

Evaluated by Western Blotting in human kidney tissue lysate.

Western Blotting Analysis: 2.0 µg/mL of this antibody detected Nephrin in 10 µg of human kidney tissue lysate.

目标描述

~185 kDa observed. Target band size appears larger than the calculated molecular weights of 132.5 kDa (isoform 1) and 128.3 kDa (isoform 2; isoform Alpha) due to glycosylation. Uncharacterized band(s) may appear in some lysates.

外形

Purified mouse monoclonal IgG2aκ antibody in buffer containing 0.1 M Tris-Glycine (pH 7.4), 150 mM NaCl with 0.05% sodium azide.
Format: Purified
Protein G Purified

储存及稳定性

Stable for 1 year at 2-8°C from date of receipt.

其他说明

Concentration: Please refer to lot specific datasheet.

免责声明

Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.

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