Gaucher's disease: increase of di- and tri-peptidyl carboxypeptidase in plasma

Marked increase of methylumbelliferyl‐tetra‐N‐acetylchitotetraoside hydrolase activity in plasma from gaucher disease patients

“…Besides the increased activity of plasma acid phosphatase in Gaucher disease, there is also a well-known hyperactivity of angiotensin-converting enzyme (Liebermann and Beutler 1976;Silverstein and Friedland 1977;Van Sande et al 1986) and certain glycosidases (Ockerman and Kohlin 1969). We have confirmed these observations, since many glycosidases in Gaucher plasma have increased activity, the highest being hexosaminidase (2.5 times normal).…”

“…The samples were stored at -20°C to -35°C. Seven patients with Gaucher disease were described previously (Van Sande et al 1986). MU-TACT hydrolase activity was determined as described (Den Tandt et al 1988) or using a scaled-down micromethod, as follows: 15#1 0.25mmol/L MU-TACT, 5/A 1 mol/L sodium acetate buffer, pH 5.5, and 20#1 acidified plasma (Den Tandt et al 1974) were incubated for 15 and 30rain at 37°C.…”