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An inducible mouse model of late onset Tay-Sachs disease.

Abstract:

Mouse models of the G(M2) gangliosidoses, Tay-Sachs and Sandhoff disease, are null for the hexosaminidase alpha and beta subunits respectively. The Sandhoff (Hexb-/-) mouse has severe neurological disease and mimics the human infantile onset variant. However, the Tay-Sachs (Hexa-/-) mouse model lacks an overt phenotype as mice can partially bypass the blocked catabolic pathway and escape disease. We have investigated whether a subset of Tay-Sachs mice develop late onset disease. We have found...

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Publication status:
Published

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Publisher copy:
10.1006/nbdi.2002.0511

Authors


Jeyakumar, M More by this author
Eliott-Smith, E More by this author
Cortina-Borja, M More by this author
Reinkensmeier, G More by this author
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Journal:
Neurobiology of disease
Volume:
10
Issue:
3
Pages:
201-210
Publication date:
2002-08-05
DOI:
EISSN:
1095-953X
ISSN:
0969-9961
URN:
uuid:d2b64836-d872-43a2-b8ac-29e448fe863f
Source identifiers:
100958
Local pid:
pubs:100958

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