ID#: 16245
Caption:
This photomicrograph details the histopathologic characteristics exhibited by a fibroblast in the case of an individual with the hereditary metabolic disorder known as Hurler syndrome. Of note, was the presence of numerous metachromatic granules within the fibroblast’s cytoplasmic interior. A rare, inherited metabolic disorder, Hurler syndrome renders a person unable to break down long chains of sugar molecules called glycosaminoglycans (GAGs), formerly known as mucopolysaccharides, and is therefore, known as mucopolysaccharidosis type-I. Unable to breakdown glycosaminoglycans, due to the absence of the enzyme, alpha-L-iduronidase, GAGs accumulate in the cells of the body’s tissues, as demonstrated in this fibroblast.
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Content Provider(s): CDC/ Dr. Daniel Perl
Creation Date: 1971
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