Fibronectin

GWB-43876F

EP-5

Each vial contains 100 ?l 1 mg/ml purified antibody containing 0. 09% sodium azide. Fibronectin is a disulfide-bonded dimer of 230-250 kD. In the extracellular matrix of several connective tissues and vessels. fibronectin is present as an insoluble protein that is extensively cross-linked by interchain disulfide bonds forming high molecular mass polymers. It is most abundant during embryonic development and tissue remodelling. Fibronectin is also present at high concentrations as a soluble plasma protein. Fibronectin is organized as a linear series of repeating modules which form domains for interaction with fibronectin itself. other matrix components (e. g. collagen and heparin) and receptors on cells (e. g. integrins).

Mostly heterodimers or multimers of alternatively spliced variants connected by 2 disulfide bonds near the carboxyl ends; to a lesser extent homodimers. Interacts with FBLN1 AMBP TNR LGALS3BP and COL13A1. Interacts with FBLN7 (By similarity).

Plasma FN (soluble dimeric form) is secreted by hepatocytes. Cellular FN (dimeric or cross-linked multimeric forms) made by fibroblasts epithelial and other cell types is deposited as fibrils in the extracellular matrix. Ugl-Y1 Ugl-Y2 and Ugl-Y3 are found in urine. Developmental Stage: Ugl-Y1 Ugl-Y2 and Ugl-Y3 are present in the urine from 0 to 17 years of age.

It is not known whether both or only one of Thr-2064 and Thr-2065 are/is glycosylated.

Defects in FN1 are the cause of glomerulopathy with fibronectin deposits type 2 (GFND2) [MIM:601894]; also known as familial glomerular nephritis with fibronectin deposits or fibronectin glomerulopathy. GFND is a genetically heterogeneous autosomal dominant disorder characterized clinically by proteinuria microscopic hematuria and hypertension that leads to end-stage renal failure in the second to fifth decade of life.

Contains 2 fibronectin type-II domains.