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rdf:type |
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lifeskim:mentions |
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pubmed:issue |
9
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pubmed:dateCreated |
1998-6-8
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pubmed:abstractText |
The GM2 gangliosidoses are a group of severe, neurodegenerative conditions that include Tay-Sachs disease, Sandhoff disease, and the GM2 activator deficiency. Bone marrow transplantation (BMT) was examined as a potential treatment for these disorders using a Sandhoff disease mouse model. BMT extended the life span of these mice from approximately 4.5 mo to up to 8 mo and slowed their neurologic deterioration. BMT also corrected biochemical deficiencies in somatic tissues as indicated by decreased excretion of urinary oligosaccharides, and lower glycolipid storage and increased levels of beta-hexosaminidase activity in visceral organs. Even with neurologic improvement, neither clear reduction of brain glycolipid storage nor improvement in neuronal pathology could be detected, suggesting a complex pathogenic mechanism. Histological analysis revealed beta-hexosaminidase-positive cells in the central nervous system and visceral organs with a concomitant reduction of colloidal iron-positive macrophages. These results may be important for the design of treatment approaches for the GM2 gangliosidoses.
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pubmed:grant |
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pubmed:commentsCorrections |
http://linkedlifedata.com/resource/pubmed/commentcorrection/9576752-1348043,
http://linkedlifedata.com/resource/pubmed/commentcorrection/9576752-1954394,
http://linkedlifedata.com/resource/pubmed/commentcorrection/9576752-2650408,
http://linkedlifedata.com/resource/pubmed/commentcorrection/9576752-3100576,
http://linkedlifedata.com/resource/pubmed/commentcorrection/9576752-3278379,
http://linkedlifedata.com/resource/pubmed/commentcorrection/9576752-3279262,
http://linkedlifedata.com/resource/pubmed/commentcorrection/9576752-3547950,
http://linkedlifedata.com/resource/pubmed/commentcorrection/9576752-3707714,
http://linkedlifedata.com/resource/pubmed/commentcorrection/9576752-7550345,
http://linkedlifedata.com/resource/pubmed/commentcorrection/9576752-7582569,
http://linkedlifedata.com/resource/pubmed/commentcorrection/9576752-7590240,
http://linkedlifedata.com/resource/pubmed/commentcorrection/9576752-7760604,
http://linkedlifedata.com/resource/pubmed/commentcorrection/9576752-7760610,
http://linkedlifedata.com/resource/pubmed/commentcorrection/9576752-8083358,
http://linkedlifedata.com/resource/pubmed/commentcorrection/9576752-8159689,
http://linkedlifedata.com/resource/pubmed/commentcorrection/9576752-8423065,
http://linkedlifedata.com/resource/pubmed/commentcorrection/9576752-8515654,
http://linkedlifedata.com/resource/pubmed/commentcorrection/9576752-8601197,
http://linkedlifedata.com/resource/pubmed/commentcorrection/9576752-8896570,
http://linkedlifedata.com/resource/pubmed/commentcorrection/9576752-8971403,
http://linkedlifedata.com/resource/pubmed/commentcorrection/9576752-9103204,
http://linkedlifedata.com/resource/pubmed/commentcorrection/9576752-9120003,
http://linkedlifedata.com/resource/pubmed/commentcorrection/9576752-9302266,
http://linkedlifedata.com/resource/pubmed/commentcorrection/9576752-9399938,
http://linkedlifedata.com/resource/pubmed/commentcorrection/9576752-9399939
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pubmed:language |
eng
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pubmed:journal |
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pubmed:citationSubset |
AIM
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pubmed:chemical |
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pubmed:status |
MEDLINE
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pubmed:month |
May
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pubmed:issn |
0021-9738
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pubmed:author |
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pubmed:issnType |
Print
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pubmed:day |
1
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pubmed:volume |
101
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pubmed:owner |
NLM
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pubmed:authorsComplete |
Y
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pubmed:pagination |
1881-8
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pubmed:dateRevised |
2009-11-18
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pubmed:meshHeading |
pubmed-meshheading:9576752-Animals,
pubmed-meshheading:9576752-Behavior, Animal,
pubmed-meshheading:9576752-Bone Marrow Transplantation,
pubmed-meshheading:9576752-Brain Chemistry,
pubmed-meshheading:9576752-Cerebral Cortex,
pubmed-meshheading:9576752-Disease Models, Animal,
pubmed-meshheading:9576752-Glycolipids,
pubmed-meshheading:9576752-Longevity,
pubmed-meshheading:9576752-Mice,
pubmed-meshheading:9576752-Mice, Mutant Strains,
pubmed-meshheading:9576752-Oligosaccharides,
pubmed-meshheading:9576752-Sandhoff Disease,
pubmed-meshheading:9576752-Survival Analysis,
pubmed-meshheading:9576752-beta-N-Acetylhexosaminidases
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pubmed:year |
1998
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pubmed:articleTitle |
Bone marrow transplantation prolongs life span and ameliorates neurologic manifestations in Sandhoff disease mice.
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pubmed:affiliation |
Genetics and Biochemistry Branch, National Institute of Diabetes and Digestive and Kidney Diseases, National Institutes of Health, Bethesda, Maryland 20892, USA.
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pubmed:publicationType |
Journal Article,
Research Support, U.S. Gov't, P.H.S.,
Research Support, Non-U.S. Gov't
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