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Laurent";Progressive noradrenergic deficits in the locus coeruleus of Mecp2 deficient mice;Journal of Neuroscience Research;;1097-4547;10.1002/jnr.22312;;Methyl-CpG binding protein 2 (MeCP2) is a transcriptional regulator. Mutations in this gene cause a wide range of neurological disorders. Mecp2 deficiency has been previously associated to catecholaminergic dysfunctions leading to autonomic defects in the brainstem and the sympathoadrenergic system of the mouse. The present study was undertaken to determine if the locus coeruleus (LC)

which was terminated at 35 weeks of gestation in view of suspicion of Walker Warburg syndrome, .  et al. 0

showed a cobblestone-like lissencephaly with a succession of normal

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Inc.;;;;;;Place: United States PMID: 23526554

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but only a tendency towards reduction. Finally, .  et al. 0

we did not found any apoptotic neurons in the pons indicating that LC neurons are not dying but are more likely loosing their catecholaminergic phenotype. In conclusion

the white matter abnormalities showed a peculiar evolution from severe hypomyelination at 4 months to patchy lesions later in childhood. Taken as a whole, .  et al. 0

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