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Literature DB >> 5277074

Induced degradation of glycosaminoglycans in Hurler's and Hunter's syndromes by plasma infusion.

N Di Ferrante, B L Nichols, P V Donnelly, G Neri, R Hrgovcic, R K Berglund.

Abstract

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.

Entities: Chemical Disease Species

Mesh：
Blood Transfusion

Year: 1971 PMID： 5277074 PMCID： PMC388923 DOI： 10.1073/pnas.68.2.303

Source DB: PubMed Journal: Proc Natl Acad Sci U S A ISSN： 0027-8424 Impact factor: 11.205

32 in total

Induced degradation of glycosaminoglycans in Hurler's and Hunter's syndromes by plasma infusion.

1. THE PRESENCE OF HYALURONIDASE IN VARIOUS MAMMALIAN TISSUES.

2. Determination of 2-deoxy-2-sulfoaminohexose content of mucopolysaccharides.

3. Mucopolysaccharidosis type 3. Morphologic and biochemical studies of two siblings with Sanfilippo syndrome.

4. Periodate oxidation of acid polysaccharides inhibition by the electrostatic field of the substrate.

5. Failure to induce scurvy by ascorbic acid depletion in a patient with Hurler's syndrome.

6. Scheie and Hurler syndromes: apparent identity of the biochemical defect.

7. Vitamin A and mucopolysaccharidosis: a clinical and biochemical evaluation.

8. Increased urinary excretion of chondroitin sulfate A and C in Hunter's syndrome.

9. Lysosomal hyaluronidase from rat liver. II. Properties.

10. The effect of retinol (vitamin-A alcohol) on urinary excretion of mucopolysaccharides in the Hurler syndrome.

Review 1. Replacement therapy in the mucopolysaccharidoses.

2. The mucopolysaccharidoses.

Review 3. Inborn errors of metabolism: principles and their applications.

Review 4. Mucopolysaccharidoses and mucolipidoses.

5. Colorimetric measurement of dermatan sulphate.

6. N-acetylgalactosamine-6-sulfate sulfatase in man. Absence of the enzyme in Morquio disease.

7. The mucopolysaccharidoses: inborn errors of glycosaminoglycan catabolism.

8. Effect of leukocyte transfusion in a child with type II mucopolysaccharidosis.

9. The defect in the Hurler and Scheie syndromes: deficiency of -L-iduronidase.

10. A clinical trial of fibroblast transplantation for the treatment of mucopolysaccharidoses.