https://m2.mtmt.hu/ hun 2026-09-23 02:48 JournalArticle 36414341 ADMIN_APPROVED true Funding Agency and Grant Number: National Research Fund (National Research, Development and Innovation Office) [K134285, PD142943, FK142971, PC-II-12/2022]; National Research, Development and Innovation Office [RRF-2.3.1-21-2022-00015]; Council of Scientific and Industrial Research (CSIR) and CSIR-Central Drug Research Institute; Jnos Bolyai Research Scholarship of the Hungarian Academy of Sciences; National Research, Development, and Innovation Office [RRF-2.3.1-21-2022-00015]; Hungarian Academy of Sciences; EKP-25 university research scholarship program of the ministry for culture and innovation [EKP-25-3-II-ELTE-616]; Pheo Para Cancer Charity Funding text: This work was supported by the National Research Fund (National Research, Development and Innovation Office) grants K134285 to K.T.-V., PD142943 to A.B., and FK142971 to A.P. and the PC-II-12/2022 grant to T.I.O. from the Hungarian Academy of Sciences. This work is also supported by the EKOP-25 university research scholarship program of the ministry for culture and innovation from the source of the national research, development, and innovation fund EKOP-25-3-II-ELTE-616 to F.& Odblac;., and EKOP-25-4-II-ELTE-46 to Z.F. This paper was supported by the Janos Bolyai Research Scholarship of the Hungarian Academy of Sciences. The work was supported by the National Research, Development, and Innovation Office (PharmaLab, RRF-2.3.1-21-2022-00015). A.N. and M.A. were supported by Council of Scientific and Industrial Research (CSIR) and CSIR-Central Drug Research Institute. This study was supported by the Pheo Para Cancer Charity. 0 false 2026-09-02T14:28:56.600+0000 2026-04-22T14:03:29.021+0000 2025-11-03T18:28:58.191+0000 true 10039043 Milánkovics Róbert /api/admin/10039043 Admin true 10039043 2026-07-21T06:43:27.377+0000 2026-03-16T15:22:54.042+0000 true 10019782 Csámer Iván /api/admin/10019782 Admin true 10019782 Admin 10025544 /api/admin/10025544 Lévayné Deseő Katalin true 10025544 true 2026-05-17T16:18:36.608+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 Ősz, Fanni The Caenorhabditis elegans sdhb-1(R244H) Model Shows Characteristics of Human PPGL Tumor Cells. true true /api/journal/10003252 REVIEWED true false 10003252 false 10003252 true 1661-6596 1422-0067 Journal FOREIGN 26 20 10185 10185 23 2025 Pheochromocytomas and paragangliomas (PPGL) are classified as rare cancers but can be highly metastatic, particularly in individuals with inherited succinate dehydrogenase B (SDHB) mutations. As current therapies and the availability of SDHB-deficient animal models are both limited, we have previously constructed a nematode PPGL model, a transgenic worm carrying the R244H missense mutation equivalent to human R230H in the sdhb-1 gene. In this study, we show that R244H mutants display characteristics of PPGL tumors, such as pseudohypoxia activation and the accumulation of reactive oxygen species. The latter can be the result of compromised antioxidant machinery, as R244H mutants have reduced levels of cytosolic and mitochondrial superoxide dismutase enzymes. In addition, the expression of mitophagy markers pink-1 (PTEN-induced putative kinase) and pdr-1 (E3 ubiquitin-protein ligase parkin) were downregulated in R244H mutants, suggesting impaired mitophagy and reflecting the crucial role of mitochondrial health in PPGL pathology. Treatments by the SDH inhibitor fluopyram revealed that the SDH complex carrying the R244H mutation in subunit B displayed residual SDH activity, which was also confirmed by our structural analyses. We also observed a link between dopaminergic neuronal health and SDHB-1. 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<span class="author-name" >Akbar Mahmood<sup>*</sup> </span> ;&nbsp;&nbsp;&nbsp; <span class="author-name" mtid="10069511"><a href="/gui2/?type=authors&mode=browse&sel=10069511" target="_blank">Zsidó Balázs Zoltán (<span class="authorship-author-name">Zsidó Balázs Zoltán</span> <span class="authorAux-mtmt"> Farmakológia</span>) </a> </span> <span class="author-affil"><span title="Pécsi Tudományegyetem">PTE</span>/<span title="Általános Orvostudományi Kar">ÁOK</span>/Farmakológiai és Farmakoterápiai Intézet</span> ;&nbsp;&nbsp;&nbsp; <span class="author-name" mtid="10001657"><a href="/gui2/?type=authors&mode=browse&sel=10001657" target="_blank">Hetényi Csaba (<span class="authorship-author-name">Hetényi Csaba</span> <span class="authorAux-mtmt"> Farmakoinformatika, molekulaszerkezet</span>) </a> </span> <span class="author-affil"><span title="Pécsi Tudományegyetem">PTE</span>/<span title="Általános Orvostudományi Kar">ÁOK</span>/Farmakológiai és Farmakoterápiai Intézet</span> ;&nbsp;&nbsp;&nbsp; <span class="author-name" mtid="10000148"><a href="/gui2/?type=authors&mode=browse&sel=10000148" target="_blank">Orbán Tamás I (<span class="authorship-author-name">Orbán Tamás I.</span> <span class="authorAux-mtmt"> Molekuláris sejtbiológia</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>/Genetikai, Sejt- és Immunbiológiai Intézet; <span title="HUN-REN Természettudományi Kutatóközpont">HRN TTK</span>/<span title="Molekuláris Élettudományi Intézet">MÉI</span>/Génreguláció Kutatócsoport; <span title="HUN-REN Természettudományi Kutatóközpont">HRN TTK</span>/Molekuláris Élettudományi Intézet</span> ;&nbsp;&nbsp;&nbsp; <span class="author-name" mtid="10067874"><a href="/gui2/?type=authors&mode=browse&sel=10067874" target="_blank">Fóthi Ábel (<span class="authorship-author-name">Fóthi Ábel</span> <span class="authorAux-mtmt"> genetika</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="Biokémiai és Molekuláris Biológiai Intézet">BMBI</span>/Molekuláris Biológiai Tanszék</span> ;&nbsp;&nbsp;&nbsp; <span class="author-name" mtid="10001140"><a href="/gui2/?type=authors&mode=browse&sel=10001140" target="_blank">Kovács Gábor M (<span class="authorship-author-name">Kovács M. Gábor</span> <span class="authorAux-mtmt"> Mikológia</span>) </a> </span> <span class="author-affil"><span title="Eötvös Loránd Tudományegyetem">ELTE</span>/<span title="Természettudományi Kar">TTK</span>/<span title="Biológiai Intézet">Bio_I</span>/Növényszervezettani Tanszék</span> ;&nbsp;&nbsp;&nbsp; <span class="author-name" mtid="10020748"><a href="/gui2/?type=authors&mode=browse&sel=10020748" target="_blank">Pintye Alexandra (<span class="authorship-author-name">Pintye Alexandra</span> <span class="authorAux-mtmt"> Biológiai tudományok</span>) </a> </span> <span class="author-affil"><span title="Eötvös Loránd Tudományegyetem">ELTE</span>/<span title="Természettudományi Kar">TTK</span>/<span title="Biológiai Intézet">Bio_I</span>/Növényszervezettani Tanszék; <span title="HUN-REN Agrártudományi Kutatóközpont">HRN ATK</span>/<span title="Növényvédelmi Intézet">NÖVI</span>/Növénykórtani Osztály</span> ;&nbsp;&nbsp;&nbsp; <span class="author-name" mtid="10058721"><a href="/gui2/?type=authors&mode=browse&sel=10058721" target="_blank">Boda Attila (<span class="authorship-author-name">Boda Attila</span> <span class="authorAux-mtmt"> sejtbiológia</span>) </a> </span> <span class="author-affil"><span title="Eötvös Loránd Tudományegyetem">ELTE</span>/<span title="Természettudományi Kar">TTK</span>/<span title="Biológiai Intézet">Bio_I</span>/Anatómiai, Sejt- és Fejlődésbiológiai Tanszék</span> ;&nbsp;&nbsp;&nbsp; <span class="author-name" >Nazir Aamir </span> ;&nbsp;&nbsp;&nbsp; <span class="author-name" mtid="10048710"><a href="/gui2/?type=authors&mode=browse&sel=10048710" target="_blank">Farkas Zsolt<sup>**</sup> (<span class="authorship-author-name">Farkas Zsolt</span> <span class="authorAux-mtmt"> Genetika</span>) </a> </span> <span class="author-affil"><span title="Eötvös Loránd Tudományegyetem">ELTE</span>/<span title="Természettudományi Kar">TTK</span>/<span title="Biológiai Intézet">Bio_I</span>/Embertani Tanszék</span> ;&nbsp;&nbsp;&nbsp; <span class="author-name" mtid="10013108"><a href="/gui2/?type=authors&mode=browse&sel=10013108" target="_blank">Takács-Vellai Krisztina ✉ (<span class="authorship-author-name">Vellainé Takács Krisztina</span> <span class="authorAux-mtmt"> Genetika</span>) </a> </span> <span class="author-affil"><span title="Eötvös Loránd Tudományegyetem">ELTE</span>/<span title="Természettudományi Kar">TTK</span>/<span title="Biológiai Intézet">Bio_I</span>/Embertani Tanszék</span> </div> </div> <div class="title"><a href="/gui2/?mode=browse&params=publication;36414341" target="_blank">The Caenorhabditis elegans sdhb-1(R244H) Model Shows Characteristics of Human PPGL Tumor Cells.</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">26</span> <span class="journal-issue">20</span> <span class="page"> Paper 10185. 23 p. </span> <span class="year">(2025)</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 style="color:blue" title="10.3390/ijms262010185" target="_blank" href="https://doi.org/10.3390/ijms262010185"> DOI </a> </span> <span class="id identifier oa_none" title="none"> <a style="color:blue" title="001601727400001" target="_blank" href="https://www.webofscience.com/wos/woscc/full-record/001601727400001"> WoS </a> </span> <span class="id identifier oa_GOLD" title=" Gold "> <a style="color:black" title="245288" target="_blank" href="https://real.mtak.hu/245288"> REAL </a> </span> <span class="id identifier oa_none" title="none"> <a style="color:blue" title="105020270421" target="_blank" href="http://www.scopus.com/record/display.url?origin=inward&eid=2-s2.0-105020270421"> Scopus </a> </span> <span class="id identifier oa_none" title="none"> <a style="color:blue" title="41155475" target="_blank" href="http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=41155475&dopt=Abstract"> PubMed </a> </span> </span> <OnlyViewableByAuthor><div class="ratings"> <div class="journal-subject">Folyóirat szakterülete: Scopus - Inorganic Chemistry&nbsp;&nbsp;&nbsp;SJR indikátor:&nbsp;D1</div> <div class="journal-subject">Folyóirat szakterülete: Scopus - Organic Chemistry&nbsp;&nbsp;&nbsp;SJR indikátor:&nbsp;D1</div> <div class="journal-subject">Folyóirat szakterülete: Scopus - Spectroscopy&nbsp;&nbsp;&nbsp;SJR indikátor:&nbsp;D1</div> <div class="journal-subject">Folyóirat szakterülete: Scopus - Catalysis&nbsp;&nbsp;&nbsp;SJR indikátor:&nbsp;Q1</div> <div class="journal-subject">Folyóirat szakterülete: Scopus - Computer Science Applications&nbsp;&nbsp;&nbsp;SJR indikátor:&nbsp;Q1</div> <div class="journal-subject">Folyóirat szakterülete: Scopus - Medicine (miscellaneous)&nbsp;&nbsp;&nbsp;SJR indikátor:&nbsp;Q1</div> <div class="journal-subject">Folyóirat szakterülete: Scopus - Physical and Theoretical Chemistry&nbsp;&nbsp;&nbsp;SJR indikátor:&nbsp;Q1</div> <div class="journal-subject">Folyóirat szakterülete: Scopus - Molecular Biology&nbsp;&nbsp;&nbsp;SJR indikátor:&nbsp;Q2</div> </div></OnlyViewableByAuthor> <div class="publication-citation"> <a target="_blank" href="/api/publication?cond=citations.related;eq;36414341&sort=publishedYear,desc&sort=title"> Idézett közlemények száma: 8 </a> </div> <div class="mtid"><span class="long-pub-mtid">Közlemény: 36414341</span> | <span class="status-data status-ADMIN_APPROVED"> Admin láttamozott </span> Forrás Idéző | <span class="type-subtype">Folyóiratcikk ( Szakcikk ) </span> | <span class="pub-category">Tudományos</span> | <span class="publication-sourceOfData">PubMed XML</span> </div> <div class="funder"> (K134285), (PD142943) Támogató: Kulturális és Innovációs Minisztérium Nemzeti Kutatási Fejlesztési és Innovációs Alap, OTKA(FK142971), (PC-II-12/2022), Nemzeti Gyógyszerkutatási és Fejlesztési Laboratórium (PharmaLab)(RRF-2.3.1-21-2022-00015) Támogató: NKFIH </div> <div class="lastModified">Utolsó módosítás: 2026.04.22. 16:03 Heronyányi Zsolt (PTE ÁOK admin 5) </div> <div class="lockedBy">Központi kezelésű 2026.05.17. 18:18 Lévayné Deseő Katalin (MTMT Központi admin) </div> <pre class="comment" style="margin-top: 0; margin-bottom: 0;"><u>Megjegyzés</u>: Funding Agency and Grant Number: National Research Fund (National Research, Development and Innovation Office) [K134285, PD142943, FK142971, PC-II-12/2022]; National Research, Development and Innovation Office [RRF-2.3.1-21-2022-00015]; Council of Scientific and Industrial Research (CSIR) and CSIR-Central Drug Research Institute; Jnos Bolyai Research Scholarship of the Hungarian Academy of Scie...</pre> </div></div>