Hurler Syndrome

 

The Hurler syndrome, named after Gertrud Hurler, refers to a genetic disorder caused due to the accumulation of glycosaminoglycans (earlier called mucopolysaccharides) as a result of lack of alpha-L iduronidase enzyme that degrades mucopolysaccharides inside lysosomes, without which the body is exposed to heparin sulfate and dermatan sulfate. Also known by the name of Mucopolysaccharidosis Type I (MPS I) or Gargoylism, the symptoms of the syndrome may appear during childhood that can manifest into an early death due to organ damage. Hurler syndrome is clinically linked with the Hunter syndrome, where the latter is X-linked and the former is autosomal recessive.

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