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Literature summary for 1.3.8.6 extracted from
- Transport and distribution of 3-hydroxyglutaric acid before and during induced encephalopathic crises in a mouse model of glutaric aciduria type 1 (2008), Biochim. Biophys. Acta, 1782, 385-390.
Organism
| Organism | UniProt | Comment | Textmining |
|---|---|---|---|
| Mus musculus | Q60759 | - |
- |
Synonyms
| Synonyms | Comment | Organism |
|---|---|---|
| GCDH | - |
Mus musculus |
| glutaryl-CoA dehydrogenase | - |
Mus musculus |
General Information
| General Information | Comment | Organism |
|---|---|---|
| malfunction | injection of (3H)-labeled 3-hydroxyglutaric acid into 6 week-old Gcdh knockout mice, a model of glutaric aciduria type 1, reveal a low recovery in kidney, liver, or brain tissue that does not differ from control mice. Significant amounts of 3-hydroxyglutaric acid are found to be excreted via the intestinal tract. Exposure of knockout mice to a high protein diet leads to an encephalopathic crisis, vacuolization in the brain, and death after 4-5 days. Under these conditions, high amounts of injected 3H-3-hydroxyglutaric acid are found in kidneys of Gcdh knockout mice, whereas the radioactivity recovered in brain and blood is reduced. The data demonstrate that under conditions mimicking encephalopathic crises the blood-brain barrier appears to remain intact | Mus musculus |
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