2015
DOI: 10.1186/s13041-015-0121-2
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Differentiation of multipotent neural stem cells derived from Rett syndrome patients is biased toward the astrocytic lineage

Abstract: BackgroundRett syndrome (RTT) is one of the most prevalent neurodevelopmental disorders in females, caused by de novo mutations in the X-linked methyl CpG-binding protein 2 gene, MECP2. Although abnormal regulation of neuronal genes due to mutant MeCP2 is thought to induce autistic behavior and impaired development in RTT patients, precise cellular mechanisms underlying the aberrant neural progression remain unclear.ResultsTwo sets of isogenic pairs of either wild-type or mutant MECP2-expressing human induced … Show more

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Cited by 80 publications
(75 citation statements)
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“…Based on the neurodevelopmental literature, a global reduction in DNA methylation and MeCP2 levels might be expected to promote astrocyte differentiation, and possibly delay or decrease neurogenesis [72,89]. In this way, alcohol would be similar to DNMT inhibitor treatment, which is known to induce differentiation along the astrocyte lineage [87].…”
Section: Fasd and Dna Methylationmentioning
confidence: 99%
“…Based on the neurodevelopmental literature, a global reduction in DNA methylation and MeCP2 levels might be expected to promote astrocyte differentiation, and possibly delay or decrease neurogenesis [72,89]. In this way, alcohol would be similar to DNMT inhibitor treatment, which is known to induce differentiation along the astrocyte lineage [87].…”
Section: Fasd and Dna Methylationmentioning
confidence: 99%
“…The current study provides mass spectrometry-based quantitative proteomic data, depth of about 7000 proteins, using an earlier developed iPSC-based models involving RTT patient cells and isogenic controls [24]. We showed that changes in dendrite morphology or synaptic defects, previously associated with RTT [22, 32], already become apparent at early developmental stages. Proteins involved in immunity and metabolism, also in line with previous studies on RTT pathology [33, 34], are differentially expressed at all time points.…”
Section: Discussionmentioning
confidence: 87%
“…Here we used an iPSC-based RTT model and performed proteome analysis on iPSC-derived neuronal stem cells (NES cells) [21]. Earlier studies proved that iPSCs from RTT patients reflect disease-specific characteristics, including changes in neuronal differentiation at early stages of development [22, 23]. However, we lack knowledge on the precise molecular mechanisms underlying the progression of the disease.…”
Section: Introductionmentioning
confidence: 99%
“…Furthermore, restoration of MeCP2 in the mutant astrocytes exerted a non-cell autonomous positive effect on mutant neurons in vivo, restoring dendritic morphology and increasing levels of the VGLUT1 transporter (Lioy et al 2011). Finally, it has been shown that Mecp2 is involved the differentiation of neural progenitors into astrocytes (Andoh-Noda et al 2015). These data showed that glia, like neurons, are integral components of the neuropathology of RTT.…”
Section: Introductionmentioning
confidence: 93%