| Title |
Lipid Composition of Whole Brain and Cerebellum in Hurler Syndrome (MPS IH) Mice
|
|---|---|
| Published in |
Neurochemical Research, January 2011
|
| DOI | 10.1007/s11064-011-0400-y |
| Pubmed ID | |
| Authors |
Karie A. Heinecke, Brandon N. Peacock, Bruce R. Blazar, Jakub Tolar, Thomas N. Seyfried |
| Abstract |
Hurler syndrome (MPS IH) is caused by a mutation in the gene encoding alpha-L-iduronidase (IDUA) and leads to the accumulation of partially degraded glycosaminoglycans (GAGs). Ganglioside content is known to increase secondary to GAG accumulation. Most studies in organisms with MPS IH have focused on changes in gangliosides GM3 and GM2, without the study of other lipids. We evaluated the total lipid distribution in the whole brain and cerebellum of MPS IH (Idua⁻/⁻) and control (Idua(+/?)) mice at 6 months and at 12 months of age. The content of total sialic acid and levels of gangliosides GM3, GM2, and GD3 were greater in the whole brains of Idua⁻/⁻ mice then in Idua (+/?) mice at 12 months of age. No other significant lipid differences were found in either whole brain or in cerebellum at either age. The accumulation of ganglioside GD3 suggests that neurodegeneration occurs in the Idua⁻/⁻) mouse brain, but not to the extent seen in human MPS IH brain. |
X Demographics
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Geographical breakdown
| Country | Count | As % |
|---|---|---|
| Unknown | 1 | 100% |
Demographic breakdown
| Type | Count | As % |
|---|---|---|
| Members of the public | 1 | 100% |
Mendeley readers
The data shown below were compiled from readership statistics for 26 Mendeley readers of this research output. Click here to see the associated Mendeley record.
Geographical breakdown
| Country | Count | As % |
|---|---|---|
| United Kingdom | 1 | 4% |
| United States | 1 | 4% |
| Unknown | 24 | 92% |
Demographic breakdown
| Readers by professional status | Count | As % |
|---|---|---|
| Student > Ph. D. Student | 8 | 31% |
| Other | 4 | 15% |
| Researcher | 3 | 12% |
| Student > Postgraduate | 2 | 8% |
| Professor > Associate Professor | 2 | 8% |
| Other | 4 | 15% |
| Unknown | 3 | 12% |
| Readers by discipline | Count | As % |
|---|---|---|
| Agricultural and Biological Sciences | 11 | 42% |
| Biochemistry, Genetics and Molecular Biology | 6 | 23% |
| Medicine and Dentistry | 6 | 23% |
| Chemistry | 1 | 4% |
| Unknown | 2 | 8% |
Attention Score in Context
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All research outputs
#18,304,230
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Outputs from Neurochemical Research
#1,475
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Outputs of similar age
#161,393
of 182,131 outputs
Outputs of similar age from Neurochemical Research
#15
of 18 outputs
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