D1b. Pathway and Pathophysiology


 Mucopolysaccharidoses are caused by defective breakdown of glycosaminoglycans (GAGs)

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What are these glycosaminoglycans? They are huge molecules, they are really macromolecules. This is on the electron microscopy, where these proteins can be seen.  It is a protein core with all kinds of long disaccharide molecules  attached to this protein. So this is a protein glycan and these parts are the true glycosaminoglycans.

 

 

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