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Induced Degradation of Glycosaminoglycans in Hurler's and Hunter's Syndromes by Plasma Infusion
Author(s) -
Nicola Di Ferrante,
Buford L. Nichols,
Patricia V. Donnelly,
Giovanni Neri,
Ruzica Hrgovcic,
Robert K. Berglund
Publication year - 1971
Publication title -
proceedings of the national academy of sciences
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 5.011
H-Index - 771
eISSN - 1091-6490
pISSN - 0027-8424
DOI - 10.1073/pnas.68.2.303
Subject(s) - dermatan sulfate , glycosaminoglycan , heparan sulfate , hunter syndrome , chemistry , excretion , endocrinology , medicine , sulfate , human plasma , degradation (telecommunications) , biochemistry , chromatography , telecommunications , organic chemistry , computer science
The effects of the administration of normal human plasma to patients affected by mucopolysaccharidoses I and II (Hurler's and Hunter's syndromes) have been evaluated. The infusion was followed by a decreased urinary excretion of relatively large molecular weight glycosaminoglycans and by an increased excretion of their products of degradation. Among the latter, products of the degradation of dermatan sulfate and heparan sulfate could be demonstrated. The results indicate that normal human plasma may contain those "factors" that are involved in the normal degradation of dermatan sulfate and heparan sulfate, that are missing in the diseased states.

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