Histone acetylation, acetyltransferases, and ataxia--alteration of histone acetylation and chromatin dynamics is implicated in the pathogenesis of polyglutamine-expansion disorders.

Advances in Protein Chemistry and Structural Biology
Shaun D McCullough, Patrick A Grant

Abstract

Eukaryotic chromosomal DNA is packaged into nucleosomes to form a dynamic structure known as chromatin. The compaction of DNA within chromatin poses a unique hindrance with regards to the accessibility of the DNA to enzymes involved in replication, transcriptional regulation, and repair. The physical structure and physiological activity of chromatin are regulated through a diverse set of posttranslational modifications, histone exchange, and structural remodeling. Of the covalent chromatin modifications, the acetylation of lysine residues within histone proteins by acetyltransferase enzymes, such as GCN5, is one of the most prevalent and important steps in the regulation of chromatin function. Alteration of histone acetyltransferase activity can easily result in the dysregulation of gene transcription and ultimately the onset of a disease state. Many transcription factors contain polyglutamine regions within their primary sequence. Mutations resulting in the elongation of these polyglutamine tracts are associated with a disease family known as the polyglutamine expansion disorders. Spinocerebellar ataxia type 7 (SCA7) is one of the nine diseases that are grouped in this family and is caused by polyglutamine expansion of the ata...Continue Reading

Citations

Mar 1, 2012·Journal of Molecular Neuroscience : MN·Xin YuAnna-Lena Ström
Mar 20, 2012·Genes & Development·Gianpiero SpedaleW W M Pim Pijnappel
Oct 29, 2013·Molecular Neurodegeneration·Carlotta E DuncanLisa M Ellerby
Oct 13, 2012·Critical Reviews in Biochemistry and Molecular Biology·Amparo Galán, Susana Rodríguez-Navarro
Nov 28, 2017·Epigenomics·Hongmei LiuCaixia Guo
Sep 1, 2017·Nature Reviews. Neuroscience·Henry L PaulsonHarry T Orr
Oct 17, 2013·The Journal of Biological Chemistry·Tara L BurkePatrick A Grant
Apr 13, 2019·Nature Reviews. Disease Primers·Thomas KlockgetherHenry L Paulson
Oct 5, 2018·Frontiers in Cellular Neuroscience·Linlin WanHong Jiang

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