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The wild\u2010type and G66V mutant\u2010type CHCHD10 proteins contain intrinsically disordered regions, and therefore, their structural ensemble studies have been experiencing difficulties using conventional tools. Here, we show our results regarding the first characterization of the structural ensemble characteristics of the G66V mutant form of CHCHD10 and the first comparison of these characteristics with the structural ensemble properties of wild\u2010type CHCHD10. We find that the structural properties, potential of mean force surfaces, and principal component analysis show stark differences between these two proteins. These results are important for a better pathology, biochemistry and structural biology understanding of CHCHD10 and its G66V genetic variant and it is likely that these reported structural properties are important for designing more efficient treatments for the Jokela type of spinal muscular atrophy disease.<\/jats:p>","DOI":"10.1002\/prot.26463","type":"journal-article","created":{"date-parts":[[2023,1,10]],"date-time":"2023-01-10T09:17:10Z","timestamp":1673342230000},"page":"739-749","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":8,"title":["Effects of the Jokela type of spinal muscular atrophy\u2010related <scp>G66V<\/scp> mutation on the structural ensemble characteristics of <scp>CHCHD10<\/scp>"],"prefix":"10.1002","volume":"91","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-5105-8331","authenticated-orcid":false,"given":"Hakan","family":"Alici","sequence":"first","affiliation":[{"name":"Department of Physics, Faculty of Arts and Sciences Zonguldak Bulent Ecevit University  Zonguldak Turkey"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Vladimir N.","family":"Uversky","sequence":"additional","affiliation":[{"name":"Department of Molecular Medicine and USF Health Byrd Alzheimer's Research Institute, Morsani College of Medicine University of South Florida  Tampa Florida USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"David E.","family":"Kang","sequence":"additional","affiliation":[{"name":"Department of Pathology, School of Medicine Case Western Reserve University  Cleveland Ohio USA"},{"name":"Louis Stokes Cleveland VA Medical Center  Cleveland Ohio USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Junga Alexa","family":"Woo","sequence":"additional","affiliation":[{"name":"Department of Pathology, School of Medicine Case Western Reserve University  Cleveland Ohio 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