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Unstable repeats can be located in the coding or the non\u2010coding region of a gene. Different pathogenic mechanisms that are hypothesised to underlie the diseases are discussed. Evidence is given both from studies in simple model systems and from studies on human material and in animal models. Since somatic instability might affect the clinical outcome, this is briefly touched on. Available data and theories on the timing and mechanisms of the repeat instability itself are discussed, along with factors that have been observed to affect instability. Finally, the question of why the often harmful unstable repeats have been maintained throughout evolution is addressed.<\/jats:p>","DOI":"10.1002\/bies.080122","type":"journal-article","created":{"date-parts":[[2009,1,20]],"date-time":"2009-01-20T07:51:09Z","timestamp":1232437869000},"page":"71-83","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":121,"title":["Microsatellite repeat instability and neurological disease"],"prefix":"10.1002","volume":"31","author":[{"given":"Judith R.","family":"Brouwer","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rob","family":"Willemsen","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ben A.","family":"Oostra","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2009,1,20]]},"reference":[{"key":"e_1_2_14_2_2","doi-asserted-by":"publisher","DOI":"10.1016\/0092-8674(91)90283-5"},{"key":"e_1_2_14_3_2","doi-asserted-by":"publisher","DOI":"10.1038\/352077a0"},{"key":"e_1_2_14_4_2","doi-asserted-by":"publisher","DOI":"10.1038\/ng0492-7"},{"key":"e_1_2_14_5_2","doi-asserted-by":"publisher","DOI":"10.1146\/annurev.neuro.29.051605.113042"},{"key":"e_1_2_14_6_2","doi-asserted-by":"publisher","DOI":"10.1038\/nature05977"},{"key":"e_1_2_14_7_2","doi-asserted-by":"publisher","DOI":"10.1002\/mrdd.20010"},{"key":"e_1_2_14_8_2","doi-asserted-by":"publisher","DOI":"10.1006\/geno.1997.4867"},{"key":"e_1_2_14_9_2","doi-asserted-by":"publisher","DOI":"10.1016\/B978-012369462-1\/50020-X"},{"key":"e_1_2_14_10_2","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/ddg349"},{"key":"e_1_2_14_11_2","doi-asserted-by":"publisher","DOI":"10.1021\/ja027967i"},{"key":"e_1_2_14_12_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.bbadis.2006.05.013"},{"key":"e_1_2_14_13_2","doi-asserted-by":"publisher","DOI":"10.1016\/B978-012369462-1\/50003-X"},{"key":"e_1_2_14_14_2","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.94.14.7388"},{"key":"e_1_2_14_15_2","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.272.49.31079"},{"key":"e_1_2_14_16_2","doi-asserted-by":"publisher","DOI":"10.1093\/nar\/29.13.2766"},{"key":"e_1_2_14_17_2","doi-asserted-by":"publisher","DOI":"10.1093\/emboj\/19.17.4439"},{"key":"e_1_2_14_18_2","doi-asserted-by":"publisher","DOI":"10.1093\/nar\/24.22.4407"},{"key":"e_1_2_14_19_2","doi-asserted-by":"publisher","DOI":"10.1126\/science.1088583"},{"key":"e_1_2_14_20_2","doi-asserted-by":"publisher","DOI":"10.1111\/j.1432-0436.2006.00060.x"},{"key":"e_1_2_14_21_2","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/ddl137"},{"key":"e_1_2_14_22_2","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.57.1.127"},{"key":"e_1_2_14_23_2","doi-asserted-by":"publisher","DOI":"10.1086\/374321"},{"key":"e_1_2_14_24_2","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/awf184"},{"key":"e_1_2_14_25_2","doi-asserted-by":"publisher","DOI":"10.4161\/rna.1.2.1035"},{"key":"e_1_2_14_26_2","doi-asserted-by":"publisher","DOI":"10.1086\/386296"},{"key":"e_1_2_14_27_2","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/awh650"},{"key":"e_1_2_14_28_2","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/ddi394"},{"key":"e_1_2_14_29_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.febslet.2005.04.004"},{"key":"e_1_2_14_30_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.neuron.2007.07.020"},{"key":"e_1_2_14_31_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.neuron.2007.07.021"},{"key":"e_1_2_14_32_2","doi-asserted-by":"publisher","DOI":"10.1086\/302985"},{"key":"e_1_2_14_33_2","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/ddg298"},{"key":"e_1_2_14_34_2","doi-asserted-by":"publisher","DOI":"10.1136\/jmg.2006.042374"},{"key":"e_1_2_14_35_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0896-6273(03)00533-6"},{"key":"e_1_2_14_36_2","doi-asserted-by":"publisher","DOI":"10.1016\/B978-012369462-1\/50029-6"},{"key":"e_1_2_14_37_2","doi-asserted-by":"publisher","DOI":"10.1016\/B978-012369462-1\/50032-6"},{"key":"e_1_2_14_38_2","doi-asserted-by":"publisher","DOI":"10.1016\/B978-012369462-1\/50035-1"},{"key":"e_1_2_14_39_2","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.96.8.4604"},{"key":"e_1_2_14_40_2","first-page":"93","volume-title":"Glutamine repeats and neurodegenerative diseases: molecular aspects","author":"Ross C. 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