Mice lacking both subunits of lysosomal β–hexosaminidase display gangliosidosis and mucopolysaccharidosis

Author:

Sango Kazunori,McDonald Michael P.,Crawley Jacqueline N.,Mack Michelle L.,Tifft Cynthia J.,Skop Elisa,Starr Christopher M.,Hoffmann Alexander,Sandhoff Konrad,Suzuki Kinuko,Proia Richard L.

Publisher

Springer Science and Business Media LLC

Subject

Genetics

Reference12 articles.

1. Yamanaka, S. et al. Targeted disruption of the Hexa gene results in mice with biochemical and pathologic features of Tay-Sachs disease. Proc. Natl. Acad. Sci. USA 91, 9975–9979 (1994).

2. Sango, K. et al. Mouse models of Tay-Sachs and Sandhoff diseases differ in neurologic phenotype and ganglioside metabolism. Nature Genet. 11, 170–176 (1995).

3. Applegarth, D.A. & Bozoian, G. Mucopolysaccharide storage in organs of a patient with Sandhoff's disease. Clin. Chim. Acta 39, 269–271 (1972).

4. Sandhoff, K., Conzelmann, E., Neufeld, E.F., Kaback, M.M. & Suzuki, K. The GM2 gangliosidoses. in The Metabolic Basis of Inherited Disease 1807–1839 (McGraw-Hill, New York, 1989).

5. Gravel, R.A. et al. The GM2 gangliosidoses. in The Metabolic and Molecular Basis of Inherited Disease (eds Scriver, C.R., Beaudet, A.L, Sly, W.S. & Valle, D.)2839–2879 (McGraw-Hill, New York, 1995).

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