| pubmed-article:9576752 | rdf:type | pubmed:Citation | lld:pubmed |
| pubmed-article:9576752 | lifeskim:mentions | umls-concept:C0027854 | lld:lifeskim |
| pubmed-article:9576752 | lifeskim:mentions | umls-concept:C0005961 | lld:lifeskim |
| pubmed-article:9576752 | lifeskim:mentions | umls-concept:C0026809 | lld:lifeskim |
| pubmed-article:9576752 | lifeskim:mentions | umls-concept:C0036161 | lld:lifeskim |
| pubmed-article:9576752 | lifeskim:mentions | umls-concept:C1711300 | lld:lifeskim |
| pubmed-article:9576752 | lifeskim:mentions | umls-concept:C0376558 | lld:lifeskim |
| pubmed-article:9576752 | lifeskim:mentions | umls-concept:C2700613 | lld:lifeskim |
| pubmed-article:9576752 | pubmed:issue | 9 | lld:pubmed |
| pubmed-article:9576752 | pubmed:dateCreated | 1998-6-8 | lld:pubmed |
| pubmed-article:9576752 | 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. | lld:pubmed |
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| pubmed-article:9576752 | pubmed:language | eng | lld:pubmed |
| pubmed-article:9576752 | pubmed:journal | http://linkedlifedata.com/r... | lld:pubmed |
| pubmed-article:9576752 | pubmed:citationSubset | AIM | lld:pubmed |
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| pubmed-article:9576752 | pubmed:status | MEDLINE | lld:pubmed |
| pubmed-article:9576752 | pubmed:month | May | lld:pubmed |
| pubmed-article:9576752 | pubmed:issn | 0021-9738 | lld:pubmed |
| pubmed-article:9576752 | pubmed:author | pubmed-author:SuzukiKK | lld:pubmed |
| pubmed-article:9576752 | pubmed:author | pubmed-author:SandhoffKK | lld:pubmed |
| pubmed-article:9576752 | pubmed:author | pubmed-author:GoldsteinGG | lld:pubmed |
| pubmed-article:9576752 | pubmed:author | pubmed-author:HoffmannAA | lld:pubmed |
| pubmed-article:9576752 | pubmed:author | pubmed-author:CrawleyJ NJN | lld:pubmed |
| pubmed-article:9576752 | pubmed:author | pubmed-author:ProiaR LRL | lld:pubmed |
| pubmed-article:9576752 | pubmed:author | pubmed-author:McDonaldM PMP | lld:pubmed |
| pubmed-article:9576752 | pubmed:author | pubmed-author:TifftC JCJ | lld:pubmed |
| pubmed-article:9576752 | pubmed:author | pubmed-author:NorflusFF | lld:pubmed |
| pubmed-article:9576752 | pubmed:issnType | Print | lld:pubmed |
| pubmed-article:9576752 | pubmed:day | 1 | lld:pubmed |
| pubmed-article:9576752 | pubmed:volume | 101 | lld:pubmed |
| pubmed-article:9576752 | pubmed:owner | NLM | lld:pubmed |
| pubmed-article:9576752 | pubmed:authorsComplete | Y | lld:pubmed |
| pubmed-article:9576752 | pubmed:pagination | 1881-8 | lld:pubmed |
| pubmed-article:9576752 | pubmed:dateRevised | 2009-11-18 | lld:pubmed |
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| pubmed-article:9576752 | pubmed:year | 1998 | lld:pubmed |
| pubmed-article:9576752 | pubmed:articleTitle | Bone marrow transplantation prolongs life span and ameliorates neurologic manifestations in Sandhoff disease mice. | lld:pubmed |
| pubmed-article:9576752 | pubmed:affiliation | Genetics and Biochemistry Branch, National Institute of Diabetes and Digestive and Kidney Diseases, National Institutes of Health, Bethesda, Maryland 20892, USA. | lld:pubmed |
| pubmed-article:9576752 | pubmed:publicationType | Journal Article | lld:pubmed |
| pubmed-article:9576752 | pubmed:publicationType | Research Support, U.S. Gov't, P.H.S. | lld:pubmed |
| pubmed-article:9576752 | pubmed:publicationType | Research Support, Non-U.S. Gov't | lld:pubmed |
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