https://m2.mtmt.hu/ hun 2026-09-21 06:47 JournalArticle 33065332 VALIDATED true Department of Chemistry, Rutgers University-Newark, Newark, NJ 07102-1811, United States Department of Biochemistry, Institute of Biochemistry and Molecular Biology, Semmelweis University, Budapest, 1082, Hungary Icahn Institute for Data Science and Genomic Technology, Icahn School of Medicine at Mount Sinai, New York, NY 10029-6501, United States Export Date: 7 October 2022 Correspondence Address: Nemeria, N.S.; Department of Chemistry, United States; email: nemeria@newark.rutgers.edu Correspondence Address: Jordan, F.; Department of Chemistry, United States; email: frjordan@newark.rutgers.edu Funding details: National Institutes of Health, NIH, GM050380, R15-GM116077 Funding details: Eunice Kennedy Shriver National Institute of Child Health and Human Development, NICHD, R03HD092878, R21HD088775 Funding details: Semmelweis Egyetem, STIA-OTKA-2021, TKP2021-EGA-25 Funding details: Nemzeti Kutatási, Fejlesztési és Innovaciós Alap, NKFIA Funding text 1: Research reported in this publication was supported by the National Institutes of Health (grants GM050380 and R15-GM116077 to F.J.); Hungarian Brain Research Program 2 (2017-1.2.1-NKP-2017-00002 to Vera Adam-Vizi, Semmelweis University), STIA-OTKA-2021 grant (from the Semmelweis University, to A.A.) and TKP2021-EGA-25 grant to A.A. Project no. TKP2021-EGA-25 has been implemented with the support provided by the Ministry of Innovation and Technology of Hungary from the National Research, Development and Innovation Fund, financed under the TKP2021-EGA funding scheme; Eunice Kennedy Shriver National Institute of Child Health &Human Development of the National Institutes of Health (R03HD092878 and R21HD088775 to S.M.H). 0 false 2026-05-22T02:54:18.139+0000 2026-04-20T09:39:08.015+0000 2022-08-25T11:01:23.472+0000 true 10080205 Szalóky-Siki Ágnes /api/admin/10080205 Admin true 2026-04-21T08:27:28.262+0000 2022-08-25T11:12:59.001+0000 true 10080205 Szalóky-Siki Ágnes /api/admin/10080205 Admin true 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 Nemeria, Natalia S. Functional Versatility of the Human 2-Oxoadipate Dehydrogenase in the L-Lysine Degradation Pathway toward Its Non-Cognate Substrate 2-Oxopimelic Acid true true /api/journal/10003252 REVIEWED true false 10003252 false 10003252 true 1661-6596 1422-0067 Journal FOREIGN 23 15 8213 8213 20 2022 The human 2-oxoadipate dehydrogenase complex (OADHc) in L-lysine catabolism is involved in the oxidative decarboxylation of 2-oxoadipate (OA) to glutaryl-CoA and NADH (+H+). Genetic findings have linked the DHTKD1 encoding 2-oxoadipate dehydrogenase (E1a), the first component of the OADHc, to pathogenesis of AMOXAD, eosinophilic esophagitis (EoE), and several neurodegenerative diseases. A multipronged approach, including circular dichroism spectroscopy, Fourier Transform Mass Spectrometry, and computational approaches, was applied to provide novel insight into the mechanism and functional versatility of the OADHc. The results demonstrate that E1a oxidizes a non-cognate substrate 2-oxopimelate (OP) as well as OA through the decarboxylation step, but the OADHc was 100-times less effective in reactions producing adipoyl-CoA and NADH from the dihydrolipoamide succinyltransferase (E2o) and dihydrolipoamide dehydrogenase (E3). The results revealed that the E2o is capable of producing succinyl-CoA, glutaryl-CoA, and adipoyl-CoA. The important conclusions are the identification of: (i) the functional promiscuity of E1a and (ii) the ability of the E2o to form acyl-CoA products derived from homologous 2-oxo acids with five, six, and even seven carbon atoms. The findings add to our understanding of both the OADHc function in the L-lysine degradative pathway and of the molecular mechanisms leading to the pathogenesis associated with DHTKD1 variants. 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/api/keyword/2734493 true true Keyword 2734494 /api/keyword/2734494 true true SjrRating 11289558 /api/sjrrating/11289558 7 0.1 journal RatingType 10002 /api/ratingtype/10002 sjr true true ClassificationExternal 1604 /api/classificationexternal/1604 true 1604 true D1 DIRECT true true /api/publication/33065332 <div class="JournalArticle Publication long-list"> <div class="authors"> <img title="Forrásközlemény" 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" >Nemeria Natalia S. ✉ </span> ;&nbsp;&nbsp;&nbsp; <span class="author-name" mtid="10059307"><a href="/gui2/?type=authors&mode=browse&sel=10059307" target="_blank">Nagy Balint (<span class="authorship-author-name">Csuka Nagy Bálint</span> <span class="authorAux-mtmt"> szerkezeti biokémia</span>) </a> </span> <span class="author-affil"><span 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</span> ;&nbsp;&nbsp;&nbsp; <span class="author-name" >Jordan Frank ✉ </span> </div> </div> <div class="title"><a href="/gui2/?mode=browse&params=publication;33065332" target="_blank">Functional Versatility of the Human 2-Oxoadipate Dehydrogenase in the L-Lysine Degradation Pathway toward Its Non-Cognate Substrate 2-Oxopimelic Acid</a></div> <div> <span class="journal-title">INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES</span> <span class="journal-issn">(<a target="_blank" href="https://portal.issn.org/resource/ISSN/1661-6596">1661-6596</a> <a target="_blank" href="https://portal.issn.org/resource/ISSN/1422-0067">1422-0067</a>)</span>: <span class="journal-volume">23</span> <span class="journal-issue">15</span> <span class="page"> Paper 8213. 20 p. </span> <span class="year">(2022)</span> </div> <div class="pub-footer"> <span class="language" xmlns="http://www.w3.org/1999/html">Nyelv: Angol | </span> <span class="identifiers"> <span class="id identifier oa_GOLD" title=" Gold "> <a 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jelölt:&nbsp;11 | WoS/Scopus jelölt:&nbsp;11 | DOI jelölt:&nbsp;11 </div> <div class="publication-citation"> <a target="_blank" href="/api/publication?cond=citations.related;eq;33065332&sort=publishedYear,desc&sort=title"> Idézett közlemények száma: 4 </a> </div> <div class="mtid"><span class="long-pub-mtid">Közlemény: 33065332</span> | <span class="status-data status-VALIDATED"> Egyeztetett </span> Forrás Idéző | <span class="type-subtype">Folyóiratcikk ( Szakcikk ) </span> | <span class="pub-category">Tudományos</span> | <span class="publication-sourceOfData">WOS TAGGED</span> </div> <div class="funder"> Hungarian National Brain Research Program(2017-1.2.1-NKP-2017-00002), (STIA-OTKA-2021), (TKP2021-EGA-25) </div> <div class="lastModified">Utolsó módosítás: 2026.04.20. 11:39 Kajtár Virág Anna (SE_AOK_Biokemia_Admin5_KV, admin) </div> <pre class="comment" style="margin-top: 0; margin-bottom: 0;"><u>Megjegyzés</u>: Department of Chemistry, Rutgers University-Newark, Newark, NJ 07102-1811, United States Department of Biochemistry, Institute of Biochemistry and Molecular Biology, Semmelweis University, Budapest, 1082, Hungary Icahn Institute for Data Science and Genomic Technology, Icahn School of Medicine at Mount Sinai, New York, NY 10029-6501, United States...</pre> </div></div>