Review
doi: 10.1016/s0896-6273(03)00328-3.
Polyglutamines placed into context
Affiliations
- PMID: 12797953
- DOI: 10.1016/s0896-6273(03)00328-3
Item in Clipboard
Review
Polyglutamines placed into context
Neuron.
.
Free article
Abstract
Nine inherited neurodegenerative disorders result from polyglutamine expansions. Two recently published papers on spinocerebellar ataxia type 1, together with studies on spinobulbar muscular atrophy last year, indicate that host protein context is the key arbiter of polyglutamine disease protein toxicity. This insight may represent the most important development in the field since the recognition of nuclear inclusions or the propensity of polyglutamine to aggregate. Indeed, an intimate and inextricable relationship may exist between polyglutamine neurotoxicity and the normal interactions, domains, modifications, and functions of the respective disease proteins.
Similar articles
-
Interaction of Akt-phosphorylated ataxin-1 with 14-3-3 mediates neurodegeneration in spinocerebellar ataxia type 1.Cell. 2003 May 16;113(4):457-68. doi: 10.1016/s0092-8674(03)00349-0. Cell. 2003. PMID: 12757707
-
Interaction between mutant ataxin-1 and PQBP-1 affects transcription and cell death.Neuron. 2002 May 30;34(5):701-13. doi: 10.1016/s0896-6273(02)00697-9. Neuron. 2002. PMID: 12062018
-
Polyglutamine neurodegeneration: minding your Ps and Qs.Nat Med. 2003 Jul;9(7):825-6. doi: 10.1038/nm0703-825. Nat Med. 2003. PMID: 12835695 No abstract available.
-
Spinocerebellar ataxias caused by polyglutamine expansions.Adv Exp Med Biol. 2002;516:47-77. doi: 10.1007/978-1-4615-0117-6_3. Adv Exp Med Biol. 2002. PMID: 12611435 Review. No abstract available.
-
Toward cell specificity in SCA1.Neuron. 2002 May 30;34(5):669-70. doi: 10.1016/s0896-6273(02)00715-8. Neuron. 2002. PMID: 12062012 Review.
Cited by
-
Functional anthology of intrinsic disorder. 2. Cellular components, domains, technical terms, developmental processes, and coding sequence diversities correlated with long disordered regions.J Proteome Res. 2007 May;6(5):1899-916. doi: 10.1021/pr060393m. Epub 2007 Mar 29. J Proteome Res. 2007. PMID: 17391015 Free PMC article.
-
Getting a handle on Huntington's disease: silencing neurodegeneration.Nat Med. 2009 Mar;15(3):252-3. doi: 10.1038/nm0309-252. Nat Med. 2009. PMID: 19265826 No abstract available.
-
CTCF regulates ataxin-7 expression through promotion of a convergently transcribed, antisense noncoding RNA.Neuron. 2011 Jun 23;70(6):1071-84. doi: 10.1016/j.neuron.2011.05.027. Neuron. 2011. PMID: 21689595 Free PMC article.
-
Genetic variation in ataxia gene ATXN7 influences cerebellar grey matter volume in healthy adults.Cerebellum. 2013 Jun;12(3):390-5. doi: 10.1007/s12311-012-0423-1. Cerebellum. 2013. PMID: 23100044
-
Progressive degeneration in a new Drosophila model of Spinocerebellar Ataxia type 7.Res Sq [Preprint]. 2023 Nov 23:rs.3.rs-3592641. doi: 10.21203/rs.3.rs-3592641/v1. Res Sq. 2023. Update in: Sci Rep. 2024 Jun 21;14(1):14332. doi: 10.1038/s41598-024-65172-4 PMID: 38045332 Free PMC article. Updated. Preprint.
Publication types
MeSH terms
Substances
LinkOut - more resources
Full Text Sources
Other Literature Sources
