| First Author | Pascucci T | Year | 2002 |
| Journal | Neuroreport | Volume | 13 |
| Issue | 18 | Pages | 2561-4 |
| PubMed ID | 12499868 | Mgi Jnum | J:89662 |
| Mgi Id | MGI:3041028 | Doi | 10.1097/00001756-200212200-00036 |
| Citation | Pascucci T, et al. (2002) Deficits in brain serotonin synthesis in a genetic mouse model of phenylketonuria. Neuroreport 13(18):2561-4 |
| abstractText | Although hyperphenylalaninemia causes neurological disturbances and mental retardation, the neuropathological effects of phenylalanine excess are still poorly understood. Brain serotonin depletion may play a major role in such disturbances and is a possible target for feasible pharmacotherapies. In the present study, we investigated hyperphenylalaninemia-related brain serotonin depletion using a genetic mouse model of phenylketonuria, the Pah(enu2) mutant. Mutant mice showed severe depletion of whole brain serotonin, a mild reduction in the brain level of tryptophan, its amino acid precursor, and major deficits in the brain level of 5-hydroxytryptophan, the second rate-limiting factor in serotonin synthesis. These results suggest that interference with brain 5-hydroxytryptophan synthesis may be the major cause of serotonin deficits in hyperphenylalaninemia. |
