Biogenic amines and their metabolites are differentially affected in the <it>Mecp2</it>-deficient mouse brain
<p>Abstract</p> <p>Background</p> <p>Rett syndrome (RTT, MIM #312750) is a severe neurological disorder caused by mutations in the X-linked methyl-CpG binding protein 2 (<it>MECP2</it>) gene. Female patients are affected with an incidence of 1/15000 live bir...
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| Format: | Article |
| Language: | English |
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BMC
2011-05-01
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| Series: | BMC Neuroscience |
| Online Access: | http://www.biomedcentral.com/1471-2202/12/47 |
| _version_ | 1811281644352438272 |
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| author | Villard Laurent Ghata Adeline Panayotis Nicolas Roux Jean-Christophe |
| author_facet | Villard Laurent Ghata Adeline Panayotis Nicolas Roux Jean-Christophe |
| author_sort | Villard Laurent |
| collection | DOAJ |
| description | <p>Abstract</p> <p>Background</p> <p>Rett syndrome (RTT, MIM #312750) is a severe neurological disorder caused by mutations in the X-linked methyl-CpG binding protein 2 (<it>MECP2</it>) gene. Female patients are affected with an incidence of 1/15000 live births and develop normally from birth to 6-18 months of age before the onset of deficits in autonomic, cognitive, motor functions (stereotypic hand movements, impaired locomotion) and autistic features. Studies on <it>Mecp2 </it>mouse models, and specifically null mice, revealed morphological and functional alterations of neurons. Several functions that are regulated by bioaminergic nuclei or peripheral ganglia are impaired in the absence of <it>Mecp2</it>.</p> <p>Results</p> <p>Using high performance liquid chromatography, combined with electrochemical detection (HPLC/EC) we found that <it>Mecp2</it><sup>-/y </sup>mice exhibit an alteration of DA metabolism in the ponto-bulbar region at 5 weeks followed by a more global alteration of monoamines when the disease progresses (8 weeks). Hypothalamic measurements suggest biphasic disturbances of norepinephrine and serotonin at pathology onset (5 weeks) that were found stabilized later on (8 weeks). Interestingly, the postnatal nigrostriatal dopaminergic deficit identified previously does not parallel the reduction of the other neurotransmitters investigated. Finally, dosage in cortical samples do not suggest modification in the monoaminergic content respectively at 5 and 8 weeks of age.</p> <p>Conclusions</p> <p>We have identified that the level of catecholamines and serotonin is differentially affected in <it>Mecp2</it><sup>-/y </sup>brain areas in a time-dependent fashion.</p> |
| first_indexed | 2024-04-13T01:37:18Z |
| format | Article |
| id | doaj.art-58e770f6dc7741a9a9873c08fa716ce9 |
| institution | Directory Open Access Journal |
| issn | 1471-2202 |
| language | English |
| last_indexed | 2024-04-13T01:37:18Z |
| publishDate | 2011-05-01 |
| publisher | BMC |
| record_format | Article |
| series | BMC Neuroscience |
| spelling | doaj.art-58e770f6dc7741a9a9873c08fa716ce92022-12-22T03:08:18ZengBMCBMC Neuroscience1471-22022011-05-011214710.1186/1471-2202-12-47Biogenic amines and their metabolites are differentially affected in the <it>Mecp2</it>-deficient mouse brainVillard LaurentGhata AdelinePanayotis NicolasRoux Jean-Christophe<p>Abstract</p> <p>Background</p> <p>Rett syndrome (RTT, MIM #312750) is a severe neurological disorder caused by mutations in the X-linked methyl-CpG binding protein 2 (<it>MECP2</it>) gene. Female patients are affected with an incidence of 1/15000 live births and develop normally from birth to 6-18 months of age before the onset of deficits in autonomic, cognitive, motor functions (stereotypic hand movements, impaired locomotion) and autistic features. Studies on <it>Mecp2 </it>mouse models, and specifically null mice, revealed morphological and functional alterations of neurons. Several functions that are regulated by bioaminergic nuclei or peripheral ganglia are impaired in the absence of <it>Mecp2</it>.</p> <p>Results</p> <p>Using high performance liquid chromatography, combined with electrochemical detection (HPLC/EC) we found that <it>Mecp2</it><sup>-/y </sup>mice exhibit an alteration of DA metabolism in the ponto-bulbar region at 5 weeks followed by a more global alteration of monoamines when the disease progresses (8 weeks). Hypothalamic measurements suggest biphasic disturbances of norepinephrine and serotonin at pathology onset (5 weeks) that were found stabilized later on (8 weeks). Interestingly, the postnatal nigrostriatal dopaminergic deficit identified previously does not parallel the reduction of the other neurotransmitters investigated. Finally, dosage in cortical samples do not suggest modification in the monoaminergic content respectively at 5 and 8 weeks of age.</p> <p>Conclusions</p> <p>We have identified that the level of catecholamines and serotonin is differentially affected in <it>Mecp2</it><sup>-/y </sup>brain areas in a time-dependent fashion.</p>http://www.biomedcentral.com/1471-2202/12/47 |
| spellingShingle | Villard Laurent Ghata Adeline Panayotis Nicolas Roux Jean-Christophe Biogenic amines and their metabolites are differentially affected in the <it>Mecp2</it>-deficient mouse brain BMC Neuroscience |
| title | Biogenic amines and their metabolites are differentially affected in the <it>Mecp2</it>-deficient mouse brain |
| title_full | Biogenic amines and their metabolites are differentially affected in the <it>Mecp2</it>-deficient mouse brain |
| title_fullStr | Biogenic amines and their metabolites are differentially affected in the <it>Mecp2</it>-deficient mouse brain |
| title_full_unstemmed | Biogenic amines and their metabolites are differentially affected in the <it>Mecp2</it>-deficient mouse brain |
| title_short | Biogenic amines and their metabolites are differentially affected in the <it>Mecp2</it>-deficient mouse brain |
| title_sort | biogenic amines and their metabolites are differentially affected in the it mecp2 it deficient mouse brain |
| url | http://www.biomedcentral.com/1471-2202/12/47 |
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