NN'-diacetylchitobiase activity in Tay-Sachs disease and Sandhoff's disease.
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.
D002498:Centrifugation; D002850:Chromatography, Gel; D002855:Chromatography, Thin Layer; D006025:Glycosaminoglycans / Q000378:metabolism; D006596:Hexosaminidases / Q000378:metabolism*; D006801:Humans; D008064:Lipidoses / Q000201:enzymology*; D008099:Liver / Q000201:enzymology / Q000503:physiopathology
D006025:Glycosaminoglycans; D006596:Hexosaminidases