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Some studies have explored the influence of non\u2010causative CAG<\/jats:styled-content> repeats on the AAO<\/jats:styled-content> of SCA<\/jats:styled-content> patients. However, studies on Chinese SCA<\/jats:styled-content>1 patients regarding candidate modifier factors involved in the variability in AAO<\/jats:styled-content> are rare.<\/jats:p><\/jats:sec>Methods<\/jats:title>In all, 152 Chinese SCA<\/jats:styled-content>1 patients who were genotyped for ATXN<\/jats:styled-content>1<\/jats:italic> and nine other (CAG<\/jats:styled-content>)n<\/jats:italic><\/jats:sub>\u2010containing genes were enrolled. Regression analysis was performed to determine the effect of the expanded allele of ATXN<\/jats:styled-content>1<\/jats:italic> (linear and quadratic effects) on AAO<\/jats:styled-content>. Then, different models were used to explore the modulatory effect of nine other (CAG<\/jats:styled-content>)n<\/jats:italic><\/jats:sub>\u2010containing genes.<\/jats:p><\/jats:sec>Results<\/jats:title>Our results verified the negative effect of the expanded allele in ATXN<\/jats:styled-content>1<\/jats:italic> by regression analysis. Some (CAG<\/jats:styled-content>)n<\/jats:italic><\/jats:sub>\u2010containing genes including TBP<\/jats:styled-content><\/jats:italic>, ATN<\/jats:styled-content>1<\/jats:italic> and HTT<\/jats:styled-content><\/jats:italic> modified AAO<\/jats:styled-content> with variance ranging from 0.8% to 3.8% and tended to decrease or delay AAO<\/jats:styled-content>. However, no modifier effects of ATXN<\/jats:styled-content>2<\/jats:italic>, ATXN<\/jats:styled-content>3<\/jats:italic>, CACNA<\/jats:styled-content>1A<\/jats:italic>, ATXN<\/jats:styled-content>7<\/jats:italic>, KCNN<\/jats:styled-content>3<\/jats:italic>, RAI<\/jats:styled-content>1<\/jats:italic> and normal ATXN<\/jats:styled-content>1<\/jats:italic> alleles in trans<\/jats:italic> were detected.<\/jats:p><\/jats:sec>Conclusion<\/jats:title>By using interaction analyses, TBP<\/jats:styled-content><\/jats:italic>, ATN<\/jats:styled-content>1<\/jats:italic> and HTT<\/jats:styled-content><\/jats:italic> were determined to have modifying effects. Our study revealed that differences in modulation may be due to ethnic and geographic diversity across different populations. Furthermore, the variability of AAO<\/jats:styled-content> was not completely explained by the genetic modifiers examined here, suggesting that other genetic or environmental factors are involved in these diseases.<\/jats:p><\/jats:sec>","DOI":"10.1111\/ene.13954","type":"journal-article","created":{"date-parts":[[2019,3,20]],"date-time":"2019-03-20T06:25:11Z","timestamp":1553063111000},"page":"1130-1136","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":14,"title":["(CAG<\/scp>)n<\/i><\/sub> loci as genetic modifiers of age at onset in patients with spinocerebellar ataxia type 1 from mainland China"],"prefix":"10.1111","volume":"26","author":[{"given":"P.","family":"Wang","sequence":"first","affiliation":[{"name":"Department of Neurology Xiangya Hospital Central South University Changsha Hunan 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