{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,2]],"date-time":"2026-09-02T09:17:00Z","timestamp":1788340620133,"version":"build-2803163510"},"reference-count":36,"publisher":"Oxford University Press (OUP)","issue":"3","license":[{"start":{"date-parts":[[2026,8,24]],"date-time":"2026-08-24T00:00:00Z","timestamp":1787529600000},"content-version":"vor","delay-in-days":54,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"funder":[{"name":"Association Fran\u00e7aise Contre les Myopathies T\u00e9l\u00e9thon","award":["24167"],"award-info":[{"award-number":["24167"]}]},{"name":"Swedish Research Council grant"},{"DOI":"10.13039\/100012538","name":"Swedish Cancer Foundation","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100012538","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100004063","name":"Knut and Alice Wallenberg foundation","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100004063","id-type":"DOI","asserted-by":"publisher"}]},{"name":"Alice Wallenberg foundation"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2026,7,6]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>The LRPPRC\/SLIRP complex is a key post-transcriptional regulator of mitochondrial gene expression, stabilizing mitochondrial mRNAs and promoting their polyadenylation and translation. Mutations in LRPPRC cause mitochondrial disorders, including Leigh syndrome French-Canadian type (LSFC), primarily affecting oxidative phosphorylation. Here, we examined the RNA-binding properties of wild-type LRPPRC and three pathogenic variants (A354V, K909del, and R1276_K1300del) using electrophoretic mobility shift assays, acoustic force spectroscopy, and AlphaFold 3 modeling. All three mutations reduced intrinsic RNA binding, with R1276_K1300del showing no detectable interaction in the absence of SLIRP. Remarkably, SLIRP restored RNA binding of this mutant to near wild-type levels, likely through conformational stabilization, as supported by single-molecule and structural analyses. These findings highlight SLIRP\u2019s critical role in modulating LRPPRC function and suggest that enhancing SLIRP activity represents a potential therapeutic strategy for LRPPRC-related mitochondrial disorders.<\/jats:p>","DOI":"10.1093\/narmme\/ugag040","type":"journal-article","created":{"date-parts":[[2026,8,21]],"date-time":"2026-08-21T11:43:34Z","timestamp":1787312614000},"source":"Crossref","is-referenced-by-count":0,"title":["SLIRP differentially modulates RNA binding of pathogenic LRPPRC variants"],"prefix":"10.1093","volume":"3","author":[{"given":"Louise","family":"Lambert","sequence":"first","affiliation":[{"name":"Universit\u00e9 Clermont Auvergne, CNRS, Laboratoire de Physique de Clermont Auvergne , F-63000 Clermont-Ferrand ,","place":["France"]},{"name":"Department of Medical Biochemistry and Cell Biology, University of Gothenburg , P.O. Box 440, SE-405 30 Gothenburg ,","place":["Sweden"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ur\u0161ka","family":"Rov\u0161nik","sequence":"additional","affiliation":[{"name":"Department of Medical Biochemistry and Cell Biology, University of Gothenburg , P.O. Box 440, SE-405 30 Gothenburg ,","place":["Sweden"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1700-3999","authenticated-orcid":false,"given":"Amandine","family":"Moretton","sequence":"additional","affiliation":[{"name":"Universit\u00e9 Clermont Auvergne, CNRS, Laboratoire de Physique de Clermont Auvergne , F-63000 Clermont-Ferrand ,","place":["France"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0005-8992-7631","authenticated-orcid":false,"given":"Katarina","family":"Johansson","sequence":"additional","affiliation":[{"name":"Department of Medical Biochemistry and Cell Biology, University of Gothenburg , P.O. Box 440, SE-405 30 Gothenburg ,","place":["Sweden"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"M\u00e3d\u00e3lina","family":"S\u00e3cultanu","sequence":"additional","affiliation":[{"name":"Department of Medical Biochemistry and Cell Biology, University of Gothenburg , P.O. Box 440, SE-405 30 Gothenburg ,","place":["Sweden"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mathilde","family":"Ch\u00e9ron","sequence":"additional","affiliation":[{"name":"Universit\u00e9 Clermont Auvergne, CNRS, Laboratoire de Physique de Clermont Auvergne , F-63000 Clermont-Ferrand ,","place":["France"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ad\u00e8le","family":"Porcan","sequence":"additional","affiliation":[{"name":"Universit\u00e9 Clermont Auvergne, CNRS, Laboratoire de Physique de Clermont Auvergne , F-63000 Clermont-Ferrand ,","place":["France"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6511-6125","authenticated-orcid":false,"given":"Bertil","family":"Macao","sequence":"additional","affiliation":[{"name":"Department of Medical Biochemistry and Cell Biology, University of Gothenburg , P.O. Box 440, SE-405 30 Gothenburg ,","place":["Sweden"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Siet","family":"van\u00a0den\u00a0Wildenberg","sequence":"additional","affiliation":[{"name":"Universit\u00e9 Clermont Auvergne, CNRS, Laboratoire de Physique de Clermont Auvergne , F-63000 Clermont-Ferrand ,","place":["France"]},{"name":"Laboratoire Magmas et Volcans, Universit\u00e9 Clermont Auvergne, CNRS, IRD, OPGC , 6 av. Blaise Pascal, Aubi\u00e8re 63170 ,","place":["France"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Xuefeng","family":"Zhu","sequence":"additional","affiliation":[{"name":"Department of Medical Biochemistry and Cell Biology, University of Gothenburg , P.O. Box 440, SE-405 30 Gothenburg ,","place":["Sweden"]},{"name":"School of Basic Medical Sciences, North China University of Science and Technology , Tangshan 063210 ,","place":["China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8713-173X","authenticated-orcid":false,"given":"Maria","family":"Falkenberg","sequence":"additional","affiliation":[{"name":"Department of Medical Biochemistry and Cell Biology, University of Gothenburg , P.O. Box 440, SE-405 30 Gothenburg ,","place":["Sweden"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1054-4274","authenticated-orcid":false,"given":"G\u00e9raldine","family":"Farge","sequence":"additional","affiliation":[{"name":"Universit\u00e9 Clermont Auvergne, CNRS, Laboratoire de Physique de Clermont Auvergne , F-63000 Clermont-Ferrand ,","place":["France"]}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","published-online":{"date-parts":[[2026,8,24]]},"reference":[{"key":"2026090204362941400_B1","doi-asserted-by":"publisher","first-page":"47","DOI":"10.1146\/annurev-biochem-052621-092014","article-title":"Replication and transcription of human mitochondrial DNA","volume":"93","author":"Falkenberg","year":"2024","journal-title":"Annu Rev Biochem"},{"key":"2026090204362941400_B2","doi-asserted-by":"crossref","first-page":"309","DOI":"10.1042\/EBC20170102","article-title":"Mitochondrial transcription and translation: 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