THE DEFECT IN HURLER AND HUNTER SYNDROMES, II. DEFICIENCY OF SPECIFIC FACTORS INVOLVED IN MUCOPOLYSACCHARIDE DEGRADATION | Zendy

Joseph C. Fratantoni | Zendy; Clara W. Hall | Zendy; Elizabeth F. Neufeld | Zendy

Open Access

THE DEFECT IN HURLER AND HUNTER SYNDROMES, II. DEFICIENCY OF SPECIFIC FACTORS INVOLVED IN MUCOPOLYSACCHARIDE DEGRADATION

Author(s) -

Joseph C. Fratantoni,

Clara W. Hall,

Elizabeth F. Neufeld

Publication year - 1969

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.64.1.360

Subject(s) - glycosaminoglycan , hurler syndrome , sulfation , genotype , biochemistry , degradation (telecommunications) , chemistry , metabolism , mucopolysaccharidosis i , biology , medicine , gene , disease , telecommunications , computer science , enzyme replacement therapy

Cultured fibroblasts, derived from patients with the Hurler and Hunter syndromes, show defective degradation of sulfated mucopolysaccharide. The aberrant metabolism of Hurler cells can be corrected by secretions of fibroblasts of genotype other than Hurler, and similarly, the defect of Hunter cells can be corrected by secretions of fibroblasts of genotype other than Hunter. The active factors in these secretions, which are heat labile and associated with macromolecules, accelerate the degradation of mucopolysaccharide.

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