Journal article
Apoptotic Cell Death in Mouse Models of GM2 Gangliosidosis and Observations on Human Tay-Sachs and Sandhoff Diseases
Abstract
Tay-Sachs and Sandhoff diseases are autosomal recessive neurodegenerative diseases resulting from the inability to catabolize GM2 ganglioside by beta-hexosaminidase A (Hex A) due to mutations of the alpha subunit (Tay-Sachs disease) or beta subunit (Sandhoff disease) of Hex A. Hex B (beta beta homodimer) is also defective in Sandhoff disease. We previously developed mouse models of both diseases and showed that Hexa-/- (Tay-Sachs) mice remain …
Authors
Huang J-Q; Trasler JM; Igdoura S; Michaud J; Hanai N; Gravel RA
Journal
Human Molecular Genetics, Vol. 6, No. 11, pp. 1879–1885
Publisher
Oxford University Press (OUP)
Publication Date
October 1, 1997
DOI
10.1093/hmg/6.11.1879
ISSN
0964-6906