遺伝性ムコ多糖代謝異常症の肝におけるヘパラン硫酸のHeterogeneityに関する研究
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Structural features of heparan sulfate isolated from the livers affected by genetic mucopolysaccharidoses type IH(Hurler syndrome), II(Hunter syndrome), IIIA(Sanfilippo syndrome A type) and IV(Morquio syndrome) were studied. The following results were obtained. In Hurler, Hunter, and Sanfilippo A type syndromes, higher values of carbazole/orcinol (c/o) ratio of uronic acid, smaller amounts of hexose and sialic acid, and greater amounts of total sulfate and N-sulfate than those of normal controls were recognized. In particular a large amount of total sulfate was characteristic of Hunter syndrome. A high value of c/o ratio of uronic acid and a large amount of N-sulfate seemed to be due to the underlying deficient enzyme which was characteristic of Sanfilippo syndrome A type. In Morquio syndrome, the analytical data were similar to those of normal controls except for a small decrease of hexose and total sulfate. Therefore, heparan sulfate obtained from this syndrome seemed to be normally degraded. The molecular weight distribution of the respective heparan sulfates isolated from the genetic mucopolysaccharidoses were mainly composed of a low molecular weight as well as those of normal controls. High performance liquid chromatography of degraded products of heparan sulfates by heparitinase digestion revealed characteristic elution patterns in the respective mucopolysaccharidoses, but the qualitative analysis could not be evaluated in detail. Three disaccharides; △ Di-GlcNS, △ Di-GlcNS6S and △ Di-GlcNAc, were obtained from the degraded products of bovine kidney heparan sulfate by heparitinase digestion. The molecular weight distribution of bovine kidney heparan sulfate was composed of a relatively high molecular weight.
- 札幌医科大学の論文
- 1985-12-02
札幌医科大学 | 論文
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