mucopolysaccharidosis VII


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Related to mucopolysaccharidosis VII: mucopolysaccharidosis type III

mucopolysaccharidosis VII

An AR lysosomal storage disease of mice and men caused by β-glucuronidase deficiency, with accumulation of glycosaminoglycans–GAGs in brain and other tissues, resulting in progressive mental deterioration and death Treatment Transplanted neural progenitor cells into the cerebral ventricles of mice corrects lysosomal storage of GAGs in neurons and glia. See Beta-glucuronidase.

mucopolysaccharidosis VII

Mucopolysaccharidosis due to a deficiency of ß-glucuronidase. Clinically, MPS VII is quite similar to MPS IH, except intelligence may be normal.
Synonym: Sly disease; glucuronidase deficiency disease; Sly syndrome
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