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Fibronectin glomerulopathy

Brad H Rovin, MD
Section Editors
Richard J Glassock, MD, MACP
Fernando C Fervenza, MD, PhD
Deputy Editor
Albert Q Lam, MD


Several glomerular disorders result from the deposition or accumulation of proteins within glomeruli that disrupt glomerular structure and function. One of these disorders is fibronectin glomerulopathy, which is a rare, familial glomerular disease caused by the deposition of fibronectin and other matrix-type proteins in glomeruli. Ultrastructurally, the deposits are electron-dense and usually homogenous to finely granular, occasionally showing fibrillar substructure.

Fibronectin glomerulopathy will be reviewed here. Other glomerular disorders resulting from the deposition or accumulation of proteins within glomeruli, including amyloidosis, fibrillary glomerulonephritis, immunotactoid glomerulonephritis, and lipoprotein glomerulopathy, are presented in detail elsewhere. (See "Renal amyloidosis" and "Glomerular diseases due to nonamyloid fibrillar deposits" and "Lipoprotein glomerulopathy".)


Biology of fibronectin — Fibronectin is a large dimeric glycoprotein consisting of two similar subunits (approximately 250 kD in weight). It functions as an adhesive glycoprotein, aiding in branching morphogenesis, cellular proliferation, wound healing, phagocytosis, platelet-platelet cohesion that is mediated in part by binding to the glycoprotein IIb/IIIa receptor, and thrombus formation [1-3].

Fibronectin normally exists in a soluble or plasma form (which circulates in blood) and an insoluble or cellular form (which is seen in basement membranes and extracellular matrices).

Fibronectin deposition — Although fibronectin is found in the normal glomerular mesangial matrix, enhanced fibronectin accumulation is variably observed in several different glomerulopathies, including diabetic nephropathy and lupus nephritis [4-11]. The increase in fibronectin expression in these diseases is secondary to locally stimulated mesangial and epithelial cell production of the insoluble or cellular form.

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Literature review current through: Nov 2017. | This topic last updated: Jul 05, 2016.
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