Reduced Activity of Serum �-Glucuronidase in Marfan Syndrome

Nakashima, Yasuhide
August 1986
Angiology;Aug1986, Vol. 37 Issue 8, p576
Academic Journal
Reduced activity of serum �-glucuronidase (EC was found in three patients with Marfan syndrome, two of whom were siblings. The serum �-glu-curonidase levels in the patients ranged from 294 to 439 �g (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 �g (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 glycosaminoglycan. The correlation coefficients of serum �-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 glycosaminoglycan levels in Marfan syndrome are due to the reduction of serum �-glucuronidase, which leads to the decreased degradation of macromolecules and, subsequently, the accumulation of the glycosaminoglycans in the tissues.


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