Effects of the Amyotrophic Lateral Sclerosis-related Q108P Mutation on the Structural Ensemble Characteristics of CHCHD10

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dc.authoridCoskuner Weber, Orkid/0000-0002-0772-9350
dc.contributor.authorAlici, Hakan
dc.contributor.authorUversky, Vladimir N.
dc.contributor.authorKang, David E.
dc.contributor.authorWoo, Junga Alexa
dc.contributor.authorCoskuner-Weber, Orkid
dc.date.accessioned2025-02-20T08:42:14Z
dc.date.available2025-02-20T08:42:14Z
dc.date.issued2024
dc.departmentTürk-Alman Üniversitesien_US
dc.description.abstractIntroduction The Q108P pathological variant of the mitochondrial Coiled-Coil-Helix-Coiled-Coil-Helix Domain-Containing Protein 10 (CHCHD10) has been implicated in amyotrophic lateral sclerosis (ALS). Both the wild-type and CHCHD10Q108P proteins exhibit intrinsically disordered regions, posing challenges for structural studies with conventional experimental tools.Method This study presents the foundational characterization of the structural features of CHCHD10Q108P and compares them with those of the wild-type counterpart. We conducted multiple run molecular dynamics simulations and bioinformatics analyses.Result Our findings reveal distinct differences in structural properties, free energy surfaces, and the outputs of principal component analysis between these two proteins. These results contribute significantly to the comprehension of CHCHD10 and its Q108P variant in terms of pathology, biochemistry, and structural biology.Conclusion The reported structural properties hold promise for informing the development of more effective treatments for ALS.
dc.identifier.doi10.2174/0113892037335036241007043530
dc.identifier.issn1389-2037
dc.identifier.issn1875-5550
dc.identifier.pmid39444183
dc.identifier.scopusqualityQ3
dc.identifier.urihttps://doi.org/10.2174/0113892037335036241007043530
dc.identifier.urihttps://hdl.handle.net/20.500.12846/1583
dc.identifier.wosWOS:001343089600001
dc.identifier.wosqualityQ4
dc.indekslendigikaynakWeb of Science
dc.indekslendigikaynakPubMed
dc.language.isoen
dc.publisherBentham Science Publ Ltd
dc.relation.ispartofCurrent Protein & Peptide Science
dc.relation.publicationcategoryDiğer
dc.rightsinfo:eu-repo/semantics/closedAccess
dc.snmzKA_WOS_20250220
dc.subjectQ108Pen_US
dc.subjectgeneticsen_US
dc.subjectCHCHD10en_US
dc.subjectamyotrophic lateral sclerosisen_US
dc.subjectstructural propertiesen_US
dc.subjectmultiple run molecular dynamics simulationsen_US
dc.subjectbioinformaticsen_US
dc.titleEffects of the Amyotrophic Lateral Sclerosis-related Q108P Mutation on the Structural Ensemble Characteristics of CHCHD10
dc.typeReview

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