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Loss of MeCP2 in aminergic neurons causes cell-autonomous defects in neurotransmitter synthesis and specific behavioral abnormalities.


Rett syndrome (RTT) is characterized by specific motor, cognitive, and behavioral deficits. Because several of these abnormalities occur in other disease states associated with alterations in aminergic neurotransmitters, we investigated the contribution of such alterations to RTT pathogenesis. We found that both individuals with RTT and Mecp2-null mice have lower-than-normal levels of aminergic metabolites and content. Deleting Mecp2 from either TH-positive dopaminergic and noradrenergic neurons or PET1-positive serotonergic neurons in mice decreased corresponding neurotransmitter concentration and specific phenotypes, likely through MeCP2 regulation of rate-limiting enzymes involved in aminergic neurotransmitter production. These data support a cell-autonomous, MeCP2-dependent mechanism for the regulation of aminergic neurotransmitter synthesis contributing to unique behavioral phenotypes.

Pubmed ID: 20007372


  • Samaco RC
  • Mandel-Brehm C
  • Chao HT
  • Ward CS
  • Fyffe-Maricich SL
  • Ren J
  • Hyland K
  • Thaller C
  • Maricich SM
  • Humphreys P
  • Greer JJ
  • Percy A
  • Glaze DG
  • Zoghbi HY
  • Neul JL


Proceedings of the National Academy of Sciences of the United States of America

Publication Data

December 22, 2009

Associated Grants

  • Agency: Autism Speaks, Id: AS1622
  • Agency: NICHD NIH HHS, Id: HD024064
  • Agency: NINDS NIH HHS, Id: K08 NS052240
  • Agency: NINDS NIH HHS, Id: K08 NS052240-01
  • Agency: NINDS NIH HHS, Id: K08 NS052240-02
  • Agency: NINDS NIH HHS, Id: K08 NS052240-03
  • Agency: NINDS NIH HHS, Id: K08 NS052240-04
  • Agency: NINDS NIH HHS, Id: K08 NS052240-05
  • Agency: NIMH NIH HHS, Id: MH078678
  • Agency: NINDS NIH HHS, Id: NS052240
  • Agency: NINDS NIH HHS, Id: NS057819
  • Agency: Howard Hughes Medical Institute, Id:

Mesh Terms

  • Amines
  • Animals
  • Homovanillic Acid
  • Hydroxyindoleacetic Acid
  • Mental Disorders
  • Methyl-CpG-Binding Protein 2
  • Mice
  • Mice, Knockout
  • Neurons
  • Phenotype
  • Tryptophan Hydroxylase
  • Tyrosine 3-Monooxygenase