What is the role of glycosaminoglycans (GAGs) in the development of amyloidosis?

Updated: Nov 12, 2018
  • Author: Robert O Holmes, Jr, DO; Chief Editor: Herbert S Diamond, MD  more...
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Answer

Answer

GAGs are heteropolysaccharides composed of long, unbranched polysaccharides that contain a repeating disaccharide unit. These proteoglycans are basement membrane components intimately associated with all types of tissue amyloid deposits. Amyloidotic organs contain increased amounts of GAGs, which may be tightly bound to amyloid fibrils. Heparan sulfate and dermatan sulfate are the GAGs most often associated with amyloidosis.

Heparan sulfate and dermatan sulfate have an unknown role in amyloidogenesis. Studies of A amyloidosis (AA) and light chain amyloidosis (AL) amyloid have shown marked restriction of the heterogeneity of the glycosaminoglycan chains, suggesting that particular subclasses of heparan and dermatan sulfates are involved.

Compounds that bind to heparan sulfate proteoglycans (eg, anionic sulfonates) decrease fibril deposition in murine models of AA and have been suggested as potential therapeutic agents.


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