D1d. Pathway and Pathophysiology

What happens when GAGs can not be degraded?



This will happen. This is a normal cell with the nucleus on the left, and on the right is a skin cell of a patient with a mucopolysaccharidosis, and as can be seen the lysosomal compartment in the cells swell tremendously. This will of course interfere with the normal biological function of the cell itself, and of the tissue and the organ. This is basically the start of the pathophysiological process in the mucopolysaccharidosis.







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