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Reduced Activity of Serum ß-Glucuronidase in Marfan Syndrome

2nd Department of Internal Medicine, University of Occupational and Environmental Health, Kitakyushu, Japan

Reduced activity of serum ß-glucuronidase (EC 3.2.1.31) was found in three patients with Marfan syndrome, two of whom were siblings. The serum ß-glu curonidase levels in the patients ranged from 294 to 439 µg (360.7±73.2, mean±SD) of the released P-nitrophenol/100 ml of serum/hour, whereas those of 7 healthy subjects ranged from 564 to 1445 µg (836.4±287.3). This difference was statistically significant (p < 0.01).

Plasma glycosaminoglycan levels in the patients were lower than those in the healthy subjects, owing mainly to the differences in plasma high-sulfated glyco saminoglycan. The correlation coefficients of serum ß-glucuronidase activity with high-sulfated and total plasma glycosaminoglycans were 0.794 and 0.809, respectively (p<0.01 and p < 0.005, respectively).

The results suggest that the decreased plasma high-sulfated glycosaminogly can levels in Marfan syndrome are due to the reduction of serum ß-glucuroni dase, which leads to the decreased degradation of macromolecules and, subsequently, the accumulation of the glycosaminoglycans in the tissues.

Angiology, Vol. 37, No. 8, 576-580 (1986)
DOI: 10.1177/000331978603700804


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