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MRS reveals additional hexose N-acetyl resonances in the brain of a mouse model for Sandhoff disease.

Abstract:

Sandhoff disease, one of several related lysosomal storage disorders, results from the build up of N-acetyl-containing glycosphingolipids in the brain and is caused by mutations in the genes encoding the hexosaminidase beta-subunit. Affected individuals undergo progressive neurodegeneration in response to the glycosphingolipid storage. (1)H magnetic resonance spectra of perchloric acid extracts of Sandhoff mouse brain exhibited several resonances ca 2.07 ppm that were not present in the corre...

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Publication status:
Published

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Publisher copy:
10.1002/nbm.984

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Journal:
NMR in biomedicine
Volume:
18
Issue:
8
Pages:
517-526
Publication date:
2005-12-01
DOI:
EISSN:
1099-1492
ISSN:
0952-3480
Source identifiers:
131031
Language:
English
Keywords:
Pubs id:
pubs:131031
UUID:
uuid:dbf81474-6294-4213-908d-3d8327b40c74
Local pid:
pubs:131031
Deposit date:
2012-12-19

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