{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,10]],"date-time":"2026-09-10T16:56:33Z","timestamp":1789059393753,"version":"build-2803163510"},"reference-count":77,"publisher":"MDPI AG","issue":"7","license":[{"start":{"date-parts":[[2023,7,3]],"date-time":"2023-07-03T00:00:00Z","timestamp":1688342400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"name":"Cluster of Excellence and Deutsche Forschungsgemeinschaft Research Center Nanoscale Microscopy and Molecular Physiology of the Brain","award":["456368804"],"award-info":[{"award-number":["456368804"]}]},{"DOI":"10.13039\/501100001659","name":"Deutsche Forschungsgemeinschaft","doi-asserted-by":"publisher","award":["456368804"],"award-info":[{"award-number":["456368804"]}],"id":[{"id":"10.13039\/501100001659","id-type":"DOI","asserted-by":"publisher"}]},{"name":"Volkswagen Stiftung","award":["456368804"],"award-info":[{"award-number":["456368804"]}]},{"name":"Shota Rustaveli Science Foundation of Georgia","award":["456368804"],"award-info":[{"award-number":["456368804"]}]},{"name":"Open-Access-Publikationsfond of the Georg-August-Universit\u00e4t G\u00f6ttingen","award":["456368804"],"award-info":[{"award-number":["456368804"]}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Biology"],"abstract":"<jats:p>Rett syndrome (RTT) is a genetic neurodevelopmental disorder with mutations in the X-chromosomal MECP2 (methyl-CpG-binding protein 2) gene. Most patients are young girls. For 7\u201318 months after birth, they hardly present any symptoms; later they develop mental problems, a lack of communication, irregular sleep and breathing, motor dysfunction, hand stereotypies, and seizures. The complex pathology involves mitochondrial structure and function. Mecp2\u2212\/y hippocampal astrocytes show increased mitochondrial contents. Neurons and glia suffer from oxidative stress, a lack of ATP, and increased hypoxia vulnerability. This spectrum of changes demands comprehensive molecular studies of mitochondria to further define their pathogenic role in RTT. Therefore, we applied a comparative proteomic approach for the first time to study the entity of mitochondrial proteins in a mouse model of RTT. In the neocortex and hippocampus of symptomatic male mice, two-dimensional gel electrophoresis and subsequent mass-spectrometry identified various differentially expressed mitochondrial proteins, including components of respiratory chain complexes I and III and the ATP-synthase FoF1 complex. The NADH-ubiquinone oxidoreductase 75 kDa subunit, NADH dehydrogenase [ubiquinone] iron-sulfur protein 8, NADH dehydrogenase [ubiquinone] flavoprotein 2, cytochrome b-c1 complex subunit 1, and ATP synthase subunit d are upregulated either in the hippocampus alone or both the hippocampus and neocortex of Mecp2\u2212\/y mice. Furthermore, the regulatory mitochondrial proteins mitofusin-1, HSP60, and 14-3-3 protein theta are decreased in the Mecp2\u2212\/y neocortex. The expressional changes identified provide further details of the altered mitochondrial function and morphology in RTT. They emphasize brain-region-specific alterations of the mitochondrial proteome and support the notion of a metabolic component of this devastating disorder.<\/jats:p>","DOI":"10.3390\/biology12070956","type":"journal-article","created":{"date-parts":[[2023,7,4]],"date-time":"2023-07-04T01:47:14Z","timestamp":1688435234000},"page":"956","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":9,"title":["Mitochondrial Proteome Changes in Rett Syndrome"],"prefix":"10.3390","volume":"12","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-6274-9047","authenticated-orcid":false,"given":"Gocha","family":"Golubiani","sequence":"first","affiliation":[{"name":"Institut f\u00fcr Neuro- und Sinnesphysiologie, Georg-August Universit\u00e4t G\u00f6ttingen, Universit\u00e4tsmedizin G\u00f6ttingen, D-37073 G\u00f6ttingen, Germany"},{"name":"Institute of Chemical Biology, Ilia State University, Tbilisi 0162, Georgia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Laura","family":"van Agen","sequence":"additional","affiliation":[{"name":"Institut f\u00fcr Neuro- und Sinnesphysiologie, Georg-August Universit\u00e4t G\u00f6ttingen, Universit\u00e4tsmedizin G\u00f6ttingen, D-37073 G\u00f6ttingen, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lia","family":"Tsverava","sequence":"additional","affiliation":[{"name":"Institute of Chemical Biology, Ilia State University, Tbilisi 0162, Georgia"},{"name":"Ivane Beritashvili Centre of Experimental Biomedicine, Tbilisi 0160, Georgia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Revaz","family":"Solomonia","sequence":"additional","affiliation":[{"name":"Institute of Chemical Biology, Ilia State University, Tbilisi 0162, Georgia"},{"name":"Ivane Beritashvili Centre of Experimental Biomedicine, Tbilisi 0160, Georgia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michael","family":"M\u00fcller","sequence":"additional","affiliation":[{"name":"Institut f\u00fcr Neuro- und Sinnesphysiologie, Georg-August Universit\u00e4t G\u00f6ttingen, Universit\u00e4tsmedizin G\u00f6ttingen, D-37073 G\u00f6ttingen, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2023,7,3]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"471","DOI":"10.1002\/ana.410140412","article-title":"A progressive syndrome of autism, dementia, ataxia, and loss of purposeful hand use in girls: Rett\u2019s syndrome: Report of 35 cases","volume":"14","author":"Hagberg","year":"1983","journal-title":"Ann. 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