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Neurochemical Characterization of Canine α ‐L‐Iduronidase Deficiency Disease (Model of Human Mucopolysaccharidosis I)
Author(s) -
Constantopoulos George,
Shull Robert M.,
Hastings Nancy,
Neufeld Elizabeth F.
Publication year - 1985
Publication title -
journal of neurochemistry
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.75
H-Index - 229
eISSN - 1471-4159
pISSN - 0022-3042
DOI - 10.1111/j.1471-4159.1985.tb05544.x
Subject(s) - dermatan sulfate , glycosaminoglycan , neurochemical , mucopolysaccharidosis i , heparan sulfate , hurler syndrome , endocrinology , medicine , mucopolysaccharidosis , mucopolysaccharidosis type i , brainstem , chemistry , biochemistry , biology , disease , enzyme replacement therapy
This report presents the neurochemical findings on the first dog to die with deficiency of α ‐L‐iduronidase (mucopolysaccharide α ‐L‐iduronohydrolase; EC 3.2.1.76). The principal findings were (a) markedly increased glycosaminoglycan content in all neural tissues examined (from threefold in sciatic nerve to 15‐fold in brainstem), (b) a modest increase in levels of gangliosides G M2 , G M3 , and G D3 , particularly in gray matter, (c) excessive accumulation of glycosaminoglycans in the CSF, (d) the increased glycosaminoglycans were dermatan sulfate and heparan sulfate, and (e) the molecular weights of the liver glycosaminoglycans were shifted toward smaller sizes, indicating partial degradation. The canine disorder thus resembles mucopolysaccharidosis I in all aspects.