PMID- 25309341 OWN - NLM STAT- PubMed-not-MEDLINE DCOM- 20141013 LR - 20211021 IS - 1662-5129 (Print) IS - 1662-5129 (Electronic) IS - 1662-5129 (Linking) VI - 8 DP - 2014 TI - Dendritic spine dysgenesis in Rett syndrome. PG - 97 LID - 10.3389/fnana.2014.00097 [doi] LID - 97 AB - Spines are small cytoplasmic extensions of dendrites that form the postsynaptic compartment of the majority of excitatory synapses in the mammalian brain. Alterations in the numerical density, size, and shape of dendritic spines have been correlated with neuronal dysfunction in several neurological and neurodevelopmental disorders associated with intellectual disability, including Rett syndrome (RTT). RTT is a progressive neurodevelopmental disorder associated with intellectual disability that is caused by loss of function mutations in the transcriptional regulator methyl CpG-binding protein 2 (MECP2). Here, we review the evidence demonstrating that principal neurons in RTT individuals and Mecp2-based experimental models exhibit alterations in the number and morphology of dendritic spines. We also discuss the exciting possibility that signaling pathways downstream of brain-derived neurotrophic factor (BDNF), which is transcriptionally regulated by MeCP2, offer promising therapeutic options for modulating dendritic spine development and plasticity in RTT and other MECP2-associated neurodevelopmental disorders. FAU - Xu, Xin AU - Xu X AD - Department of Neurobiology, Civitan International Research Center, The University of Alabama at Birmingham, Birmingham, AL USA. FAU - Miller, Eric C AU - Miller EC AD - Department of Neurobiology, Civitan International Research Center, The University of Alabama at Birmingham, Birmingham, AL USA. FAU - Pozzo-Miller, Lucas AU - Pozzo-Miller L AD - Department of Neurobiology, Civitan International Research Center, The University of Alabama at Birmingham, Birmingham, AL USA. LA - eng GR - R01 NS065027/NS/NINDS NIH HHS/United States GR - R21 HD074418/HD/NICHD NIH HHS/United States PT - Journal Article PT - Review DEP - 20140910 PL - Switzerland TA - Front Neuroanat JT - Frontiers in neuroanatomy JID - 101477943 PMC - PMC4159975 OTO - NOTNLM OT - BDNF OT - MeCP2 OT - TrkB OT - autism spectrum disorder OT - excitatory synapse OT - hippocampus OT - organotypic slice cultures OT - spine density EDAT- 2014/10/14 06:00 MHDA- 2014/10/14 06:01 PMCR- 2014/01/01 CRDT- 2014/10/14 06:00 PHST- 2014/07/19 00:00 [received] PHST- 2014/08/25 00:00 [accepted] PHST- 2014/10/14 06:00 [entrez] PHST- 2014/10/14 06:00 [pubmed] PHST- 2014/10/14 06:01 [medline] PHST- 2014/01/01 00:00 [pmc-release] AID - 10.3389/fnana.2014.00097 [doi] PST - epublish SO - Front Neuroanat. 2014 Sep 10;8:97. doi: 10.3389/fnana.2014.00097. eCollection 2014.