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TitleStructural insight and characterization of human Twinkle helicase in mitochondrial disease.
Journal, issue, pagesProc Natl Acad Sci U S A, Vol. 119, Issue 32, Page e2207459119, Year 2022
Publish dateAug 9, 2022
AuthorsAmanda A Riccio / Jonathan Bouvette / Lalith Perera / Matthew J Longley / Juno M Krahn / Jason G Williams / Robert Dutcher / Mario J Borgnia / William C Copeland /
PubMed AbstractTwinkle is the mammalian helicase vital for replication and integrity of mitochondrial DNA. Over 90 Twinkle helicase disease variants have been linked to progressive external ophthalmoplegia and ...Twinkle is the mammalian helicase vital for replication and integrity of mitochondrial DNA. Over 90 Twinkle helicase disease variants have been linked to progressive external ophthalmoplegia and ataxia neuropathies among other mitochondrial diseases. Despite the biological and clinical importance, Twinkle represents the only remaining component of the human minimal mitochondrial replisome that has yet to be structurally characterized. Here, we present 3-dimensional structures of human Twinkle W315L. Employing cryo-electron microscopy (cryo-EM), we characterize the oligomeric assemblies of human full-length Twinkle W315L, define its multimeric interface, and map clinical variants associated with Twinkle in inherited mitochondrial disease. Cryo-EM, crosslinking-mass spectrometry, and molecular dynamics simulations provide insight into the dynamic movement and molecular consequences of the W315L clinical variant. Collectively, this ensemble of structures outlines a framework for studying Twinkle function in mitochondrial DNA replication and associated disease states.
External linksProc Natl Acad Sci U S A / PubMed:35914129 / PubMed Central
MethodsEM (single particle)
Resolution3.8 - 4.5 Å
Structure data

25743

7t8b

EMDB-25743, PDB-7t8b:
Octameric Human Twinkle Helicase Clinical Variant W315L
Method: EM (single particle) / Resolution: 3.8 Å

25744

7t8c

EMDB-25744, PDB-7t8c:
Heptameric Human Twinkle Helicase Clinical Variant W315L
Method: EM (single particle) / Resolution: 4.5 Å

EMDB-25746: Octameric Twinkle Helicase Clinical Variant W315L, local refinement
Method: EM (single particle) / Resolution: 3.9 Å

Source
  • homo sapiens (human)
KeywordsDNA BINDING PROTEIN / Helicase / walker A / walker B / DNA binding

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