Abstract

Two types of GM 1 gangliosidosis were studied biochemically. Type 1 liver accumulated non-lipid hexosamine in addition to GM 1 ganglioside, but there was no increase of hexosamine and GM 1 in type 2 liver. The optimum pH of liver beta-galactosidase of type 1 and type 2 was 5–6 while that of the normal control was 4.5. Type 1 brain beta-galactosidase showed a slightly acidic optimum pH, i.e. 4.0 in comparison with that of the normal control. The optimum pH of type 2 brain beta-galactosidase was 5.5, like the liver enzyme. Thermostability of liver beta-galactosidase was the same in type 1 and type 2, while that of brain was different. Beta-galactosidase of type 1 and type 2 liver is more stable at 42 °C than the normal control, but a different thermostability was observed in type 1 and type 2 brain. Liver beta-galactosidase of type 1 showed one peak each at acid and neutral pH, and type 2 liver had only one peak at neutral pH.

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