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. 1974 Aug;141(2):597-9.
doi: 10.1042/bj1410597.

NN'-diacetylchitobiase activity in Tay-Sachs disease and Sandhoff's disease

J L Stirling

NN'-diacetylchitobiase activity in Tay-Sachs disease and Sandhoff's disease

J L Stirling. Biochem J. 1974 Aug.

Abstract

In Tay-Sachs disease and Sandhoff's disease respectively, one of the N-acetyl-beta-hexosaminidases (form A) or both (forms A and B) are absent, but glycosaminoglycans containing N-acetylhexosamines are not accumulated. The presence of NN'-diacetylchitobiase in livers from patients with these diseases raises the possibility that this new enzyme is involved in the metabolism of glycosaminoglycans rather than the enzymes previously described.

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