scispace - formally typeset
Open AccessJournal ArticleDOI

From neural development to cognition: unexpected roles for chromatin

Jehnna L. Ronan, +2 more
- 01 May 2013 - 
- Vol. 14, Iss: 5, pp 347-359
TLDR
Interestingly, mutations in EZH2 and certain BAF complex components have roles in both neurodevelopmental disorders and cancer, and overlapping point mutations are suggesting functionally important residues and domains.
Abstract
Recent genome-sequencing studies in human neurodevelopmental and psychiatric disorders have revealed mutations in chromatin-modifying enzymes, such as chromatin remodellers and histone-modifying enzymes. Such studies are improving our understanding of the roles of these modifiers in human neurodevelopment, and this article discusses the emerging roles for several of these enzymes in development and disease.

read more

Content maybe subject to copyright    Report

Citations
More filters
Journal ArticleDOI

A de novo variant of CHD8 in a patient with autism spectrum disorder.

TL;DR: The findings elaborate the genotype-phenotype correlation and confirm that the CHD8 disruptions represent a clinical ASD subtype and further highlight the significance of implementing genomic medicine in clinical practice for an early intervention and necessary support for the families.
Journal ArticleDOI

Central nervous system-specific knockout of Brg1 causes growth retardation and neuronal degeneration.

TL;DR: The effects of conditional knockout of the Brahma-related gene-1 (Brg1) in the mouse central nervous system on postnatal development were assessed to provide an insight in chromatin remodeling regulation in postnatal neuronal development.
Journal ArticleDOI

Akirin proteins in development and disease: critical roles and mechanisms of action

TL;DR: Data support the hypothesis that Akirins act as a “bridge” between a variety of transcription factors and major chromatin remodeling complexes, and discuss several important questions remaining to be addressed.
Journal ArticleDOI

CRISPR-engineered genome editing for the next generation neurological disease modeling.

TL;DR: Recent progress of CRISPR‐based brain disease modeling studies are reviewed and future requirement to tackle current difficulties in usage of these technologies are discussed.
Journal ArticleDOI

Strength of functional signature correlates with effect size in autism.

TL;DR: A convergent functional signal is seen for a subset of known and novel functions in ASD from all sources of genetic variation, and meta-analytic approaches explicitly accounting for different study designs can be adapted to other diseases to discover novel functional associations and increase statistical power.
References
More filters
Journal ArticleDOI

An integrated encyclopedia of DNA elements in the human genome

TL;DR: The Encyclopedia of DNA Elements project provides new insights into the organization and regulation of the authors' genes and genome, and is an expansive resource of functional annotations for biomedical research.
Journal ArticleDOI

Translating the Histone Code

TL;DR: It is proposed that this epigenetic marking system represents a fundamental regulatory mechanism that has an impact on most, if not all, chromatin-templated processes, with far-reaching consequences for cell fate decisions and both normal and pathological development.
Journal ArticleDOI

Functions of DNA methylation: islands, start sites, gene bodies and beyond

TL;DR: Improved genome-scale mapping of methylation allows us to evaluate DNA methylation in different genomic contexts: transcriptional start sites with or without CpG islands, in gene bodies, at regulatory elements and at repeat sequences.
Journal ArticleDOI

Rett syndrome is caused by mutations in X-linked MECP2, encoding methyl-CpG-binding protein 2.

TL;DR: This study reports the first disease-causing mutations in RTT and points to abnormal epigenetic regulation as the mechanism underlying the pathogenesis of RTT.
Related Papers (5)

Patterns and rates of exonic de novo mutations in autism spectrum disorders

Synaptic, transcriptional and chromatin genes disrupted in autism

Silvia De Rubeis, +99 more
- 13 Nov 2014 -