https://m2.mtmt.hu/ hun 2026-05-30 13:45 JournalArticle 3407990 VALIDATED true Department of Medical Biochemistry, MTA-SE Laboratory for Neurobiochemistry, Semmelweis University, Budapest, H-1094, Hungary Macromolecular Crystallography, Helmholtz-Zentrum Berlin für Materialien und Energie, D-12489, Berlin, Germany Cited By :2 Export Date: 24 January 2020 CODEN: FRBME Correspondence Address: Ambrus, A.; Department of Medical Biochemistry, Semmelweis University, 37-47 Tuzolto Street, Hungary; email: ambrus.attila@med.semmelweis-univ.hu Chemicals/CAS: dihydrolipoamide dehydrogenase, 37340-89-9, 9001-18-7; Dihydrolipoamide Dehydrogenase; Mutant Proteins Funding details: Hungarian Scientific Research Fund Funding details: Hungarian Scientific Research Fund, 112230 Funding details: Magyar Tudományos Akadémia, 02001 Funding text 1: Financial support was secured from the Hungarian Academy of Sciences (MTA grant 02001 to V.A-V.), the Hungarian Scientific Research Fund (OTKA grant 112230 to V.A-V.), the Hungarian Brain Research Program (grants KTIA_13_NAP-A-III/6. and 2017-1.2.1-NKP-2017-00002 to V.A-V.), the EMBO , Fulbright , and Bolyai Fellowships (to A.A.), and the Young Investigator Research Grants of the Semmelweis University and Gedeon Richter Pharmaceuticals PIc . (to A.A.). Data collection at Helmholtz-Zentrum Berlin was carried out under contract number 16204087-ST. Department of Medical Biochemistry, MTA-SE Laboratory for Neurobiochemistry, Semmelweis University, Budapest, H-1094, Hungary Macromolecular Crystallography, Helmholtz-Zentrum Berlin für Materialien und Energie, D-12489, Berlin, Germany Cited By :3 Export Date: 4 January 2021 CODEN: FRBME Correspondence Address: Ambrus, A.; Department of Medical Biochemistry, Semmelweis University, 37-47 Tuzolto Street, Hungary; email: ambrus.attila@med.semmelweis-univ.hu Chemicals/CAS: dihydrolipoamide dehydrogenase, 37340-89-9, 9001-18-7; Dihydrolipoamide Dehydrogenase; Mutant Proteins Funding details: Hungarian Scientific Research Fund, OTKA Funding details: Magyar Tudományos Akadémia, MTA Funding details: Hungarian Scientific Research Fund, OTKA, 112230 Funding details: Magyar Tudományos Akadémia, MTA, 02001 Funding text 1: Financial support was secured from the Hungarian Academy of Sciences (MTA grant 02001 to V.A-V.), the Hungarian Scientific Research Fund (OTKA grant 112230 to V.A-V.), the Hungarian Brain Research Program (grants KTIA_13_NAP-A-III/6. and 2017-1.2.1-NKP-2017-00002 to V.A-V.), the EMBO , Fulbright , and Bolyai Fellowships (to A.A.), and the Young Investigator Research Grants of the Semmelweis University and Gedeon Richter Pharmaceuticals PIc . (to A.A.). Data collection at Helmholtz-Zentrum Berlin was carried out under contract number 16204087-ST. Department of Medical Biochemistry, MTA-SE Laboratory for Neurobiochemistry, Semmelweis University, Budapest, H-1094, Hungary Macromolecular Crystallography, Helmholtz-Zentrum Berlin für Materialien und Energie, D-12489, Berlin, Germany Cited By :3 Export Date: 5 January 2021 CODEN: FRBME Correspondence Address: Ambrus, A.; Department of Medical Biochemistry, Semmelweis University, 37-47 Tuzolto Street, Hungary; email: ambrus.attila@med.semmelweis-univ.hu Chemicals/CAS: dihydrolipoamide dehydrogenase, 37340-89-9, 9001-18-7; Dihydrolipoamide Dehydrogenase; Mutant Proteins Funding details: Hungarian Scientific Research Fund, OTKA Funding details: Magyar Tudományos Akadémia, MTA Funding details: Hungarian Scientific Research Fund, OTKA, 112230 Funding details: Magyar Tudományos Akadémia, MTA, 02001 Funding text 1: Financial support was secured from the Hungarian Academy of Sciences (MTA grant 02001 to V.A-V.), the Hungarian Scientific Research Fund (OTKA grant 112230 to V.A-V.), the Hungarian Brain Research Program (grants KTIA_13_NAP-A-III/6. and 2017-1.2.1-NKP-2017-00002 to V.A-V.), the EMBO , Fulbright , and Bolyai Fellowships (to A.A.), and the Young Investigator Research Grants of the Semmelweis University and Gedeon Richter Pharmaceuticals PIc . (to A.A.). Data collection at Helmholtz-Zentrum Berlin was carried out under contract number 16204087-ST. 0 2018-08-30T22:20:07.000+0000 false 3407990 2026-05-18T12:31:41.260+0000 2021-03-31T08:28:03.120+0000 2018-08-30T07:39:35.000+0000 true 10060658 Szabó Eszter /api/author/10060658 Author true 10060658 2026-04-21T08:48:49.422+0000 2019-01-07T13:48:12.631+0000 true 10045364 Fi Tamás /api/admin/10045364 Admin true 10045364 2019-12-09T08:07:19.035+0000 true 10026589 Tichy Rita /api/admin/10026589 Admin true 10026589 Admin 10003134 /api/admin/10003134 Wolf György true 10003134 true 2021-04-07T11:25:08.372+0000 true false true 24 24 /api/publicationtype/24 PublicationType 1 true 24 JournalArticle true 10000059 Article true 24 24 /api/publicationtype/24 PublicationType 1 true 24 JournalArticle /api/subtype/10000059 Szakcikk SubType 101 true 10000059 true 1 /api/category/1 Category true 1 Szabo, E Crystal structures of the disease-causing D444V mutant and the relevant wild type human dihydrolipoamide dehydrogenase true true /api/journal/1638 REVIEWED true false 1638 false 1638 true 0891-5849 1873-4596 Journal FOREIGN 124 214 220 214 7 2018 We report the crystal structures of the human (dihydro)lipoamide dehydrogenase (hLADH, hE3) and its disease-causing homodimer interface mutant D444V-hE3 at 2.27 and 1.84 Å resolution, respectively. The wild type structure is a unique uncomplexed, unliganded hE3 structure with the true canonical sequence. Based on the structural information a novel molecular pathomechanism is proposed for the impaired catalytic activity and enhanced capacity for reactive oxygen species generation of the pathogenic mutant. 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style="float: left" src="/frontend/resources/grid/publication-core-icon.png"> <img title="Idézőközlemény" style="float: left" src="/frontend/resources/grid/publication-citation-icon.png"> <div class="autype autype0"> <span class="author-name" mtid="10060658"><a href="/gui2/?type=authors&mode=browse&sel=10060658" target="_blank">Szabo E (<span class="authorship-author-name">Szabó Eszter</span> <span class="authorAux-mtmt"> biokémia</span>) </a> </span> <span class="author-affil"><span title="Semmelweis Egyetem">SE</span>/<span title="Általános Orvostudományi Kar">AOK</span>/<span title="Intézet">I</span>/<span title="Orvosi Biokémiai Intézet">OBI</span>/MTA-SE Neurobiokémiai Kutatócsoport</span> ;&nbsp;&nbsp;&nbsp; <span class="author-name" mtid="10021946"><a href="/gui2/?type=authors&mode=browse&sel=10021946" target="_blank">Mizsei R (<span class="authorship-author-name">Mizsei Réka</span> <span class="authorAux-mtmt"> szerkezeti biokémia</span>) </a> </span> <span class="author-affil"><span title="Semmelweis Egyetem">SE</span>/<span title="Általános Orvostudományi Kar">AOK</span>/<span title="Intézet">I</span>/Orvosi Biokémiai Intézet</span> ;&nbsp;&nbsp;&nbsp; <span class="author-name" >Wilk P </span> ;&nbsp;&nbsp;&nbsp; <span class="author-name" mtid="10060538"><a href="/gui2/?type=authors&mode=browse&sel=10060538" target="_blank">Zambo Z (<span class="authorship-author-name">Zámbó Zsófia Melinda</span> <span class="authorAux-mtmt"> biokémia</span>) </a> </span> <span class="author-affil"><span title="Semmelweis Egyetem">SE</span>/<span title="Általános Orvostudományi Kar">AOK</span>/<span title="Intézet">I</span>/Orvosi Biokémiai Intézet</span> ;&nbsp;&nbsp;&nbsp; <span class="author-name" mtid="10003430"><a href="/gui2/?type=authors&mode=browse&sel=10003430" target="_blank">Torocsik B (<span class="authorship-author-name">Törőcsik Beáta</span> <span class="authorAux-mtmt"> orvosi biokémia</span>) </a> </span> <span class="author-affil"><span title="Semmelweis Egyetem">SE</span>/<span title="Általános Orvostudományi Kar">AOK</span>/<span title="Intézet">I</span>/<span title="Orvosi Biokémiai Intézet">OBI</span>/MTA-SE Neurobiokémiai Kutatócsoport</span> ;&nbsp;&nbsp;&nbsp; <span class="author-name" >Weiss MS </span> ;&nbsp;&nbsp;&nbsp; <span class="author-name" mtid="10000397"><a href="/gui2/?type=authors&mode=browse&sel=10000397" target="_blank">Adam-Vizi V (<span class="authorship-author-name">Ádám Veronika</span> <span class="authorAux-mtmt"> idegtudomány, biokémia</span>) </a> </span> <span class="author-affil"><span title="Semmelweis Egyetem">SE</span>/<span title="Általános Orvostudományi Kar">AOK</span>/<span title="Intézet">I</span>/<span title="Orvosi Biokémiai Intézet">OBI</span>/MTA-SE Neurobiokémiai Kutatócsoport</span> ;&nbsp;&nbsp;&nbsp; <span class="author-name" mtid="10000177"><a href="/gui2/?type=authors&mode=browse&sel=10000177" target="_blank">Ambrus A ✉ (<span class="authorship-author-name">Ambrus Attila</span> <span class="authorAux-mtmt"> Biokémia</span>) </a> </span> <span class="author-affil"><span title="Semmelweis Egyetem">SE</span>/<span title="Általános Orvostudományi Kar">AOK</span>/<span title="Intézet">I</span>/<span title="Orvosi Biokémiai Intézet">OBI</span>/MTA-SE Neurobiokémiai Kutatócsoport</span> </div> </div> <div class="title"><a href="/gui2/?mode=browse&params=publication;3407990" target="_blank">Crystal structures of the disease-causing D444V mutant and the relevant wild type human dihydrolipoamide dehydrogenase</a></div> <div> <span class="journal-title">FREE RADICAL BIOLOGY AND MEDICINE</span> <span class="journal-issn">(<a target="_blank" href="https://portal.issn.org/resource/ISSN/0891-5849">0891-5849</a> <a target="_blank" href="https://portal.issn.org/resource/ISSN/1873-4596">1873-4596</a>)</span>: <span class="journal-volume">124</span> <span class="page"> pp 214-220 </span> <span class="year">(2018)</span> </div> <div class="pub-footer"> <span 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class="publication-sourceOfData">Scopus</span> </div> <div class="lastModified">Utolsó módosítás: 2021.03.31. 10:28 xBökönyi Zita (INAKTÍV_SE_AOK_OBI_Admin5_BZ, admin) </div> <div class="lockedBy">Központi kezelésű 2021.04.07. 13:25 Wolf György (MTMT Központi admin) </div> <pre class="comment" style="margin-top: 0; margin-bottom: 0;"><u>Megjegyzés</u>: Department of Medical Biochemistry, MTA-SE Laboratory for Neurobiochemistry, Semmelweis University, Budapest, H-1094, Hungary Macromolecular Crystallography, Helmholtz-Zentrum Berlin für Materialien und Energie, D-12489, Berlin, Germany Cited By :2 Export Date: 24 January 2020 CODEN: FRBME ...</pre> </div></div>