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2023 1
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OPA1 promotes ferroptosis by augmenting mitochondrial ROS and suppressing an integrated stress response.
Liang FG, Zandkarimi F, Lee J, Axelrod JL, Pekson R, Yoon Y, Stockwell BR, Kitsis RN. Liang FG, et al. Mol Cell. 2024 Aug 22;84(16):3098-3114.e6. doi: 10.1016/j.molcel.2024.07.020. Epub 2024 Aug 13. Mol Cell. 2024. PMID: 39142278 Free PMC article.
Here, we report that human and mouse cells lacking OPA1 are markedly resistant to ferroptosis. Reconstitution with OPA1 mutants demonstrates that ferroptosis sensitization requires the GTPase activity but is independent of OPA1-mediated mitochondrial fusion. …
Here, we report that human and mouse cells lacking OPA1 are markedly resistant to ferroptosis. Reconstitution with OPA1 mutant …
The Balance of MFN2 and OPA1 in Mitochondrial Dynamics, Cellular Homeostasis, and Disease.
Zanfardino P, Amati A, Perrone M, Petruzzella V. Zanfardino P, et al. Biomolecules. 2025 Mar 18;15(3):433. doi: 10.3390/biom15030433. Biomolecules. 2025. PMID: 40149969 Free PMC article. Review.
This review explores the molecular mechanisms underlying mitochondrial fusion, the impact of MFN2 and OPA1 dysfunction on oxidative phosphorylation and autophagy, and their role in disease progression. Additionally, we discuss the divergent cellular responses to MFN2 and …
This review explores the molecular mechanisms underlying mitochondrial fusion, the impact of MFN2 and OPA1 dysfunction on oxidative p …
Clinical and genetic landscape of optic atrophy in 826 families: insights from 50 nuclear genes.
Zheng Y, Wang P, Li S, Long Y, Jiang Y, Guo D, Jia X, Liu M, Zeng Y, Xiao X, Hejtmancik JF, Zhang Q, Sun W. Zheng Y, et al. Brain. 2025 May 13;148(5):1604-1620. doi: 10.1093/brain/awae324. Brain. 2025. PMID: 39423307 Free PMC article.
The top five genes implicated in nHON in our in-house cohort were OPA1, WFS1, FDXR, ACO2 and AFG3L2, which accounted for 82.46% of probands. Although OPA1 was the most prevalent nHON-causative gene in both our cohort (53.25%) and a literature review (37.09%), the pr …
The top five genes implicated in nHON in our in-house cohort were OPA1, WFS1, FDXR, ACO2 and AFG3L2, which accounted for 82.46% of pr …
A novel MTORC2-AKT-ROS axis triggers mitofission and mitophagy-associated execution of colorectal cancer cells upon drug-induced activation of mutant KRAS.
Iskandar K, Foo J, Liew AQX, Zhu H, Raman D, Hirpara JL, Leong YY, Babak MV, Kirsanova AA, Armand AS, Oury F, Bellot G, Pervaiz S. Iskandar K, et al. Autophagy. 2024 Jun;20(6):1418-1441. doi: 10.1080/15548627.2024.2307224. Epub 2024 Feb 25. Autophagy. 2024. PMID: 38261660 Free PMC article.
Here we report that drug (merodantoin; C1)-induced activation of mutant KRAS promotes phospho-AKT S473-dependent ROS-mediated S616 phosphorylation and mitochondrial localization of DNM1L/DRP1 (dynamin 1 like) and cleavage of the fusion-associated protein OPA1 (OPA1 …
Here we report that drug (merodantoin; C1)-induced activation of mutant KRAS promotes phospho-AKT S473-dependent ROS-mediated S616 phosphory …
18-Years of single-centre DNA testing in over 7000 index cases with inherited retinal dystrophies and optic neuropathies.
Kiel C, Biasella F, Stöhr H, Rating P, Spital G, Kellner U, Hufendiek K, Huchzermeyer C, Jaegle H, Ruether K, Weber BHF. Kiel C, et al. Sci Rep. 2024 Oct 26;14(1):25529. doi: 10.1038/s41598-024-77014-4. Sci Rep. 2024. PMID: 39462066 Free PMC article.
The genes most implicated in disease pathologies are ABCA4, accounting for 23.8% of all IRD/ION index cases, followed by BEST1 (7.8%), USH2A (6.2%), PRPH2 (5.7%), RPGR (5.6%), RS1 (5.5%), OPA1 (4.3%), and RHO (3.1%). Our study has compiled the most extensive dataset in com …
The genes most implicated in disease pathologies are ABCA4, accounting for 23.8% of all IRD/ION index cases, followed by BEST1 (7.8%), USH2A …
Tetrahydrobenzimidazole TMQ0153 targets OPA1 and restores drug sensitivity in AML via ROS-induced mitochondrial metabolic reprogramming.
Park SJ, Cerella C, Kang JM, Byun J, Kum D, Orlikova-Boyer B, Lorant A, Schnekenburger M, Al-Mourabit A, Christov C, Lee J, Han BW, Diederich M. Park SJ, et al. J Exp Clin Cancer Res. 2025 Apr 7;44(1):114. doi: 10.1186/s13046-025-03372-0. J Exp Clin Cancer Res. 2025. PMID: 40197337 Free PMC article.
Effects of TMQ0153 treatment on OPA1 and mitofusin (MFN)2 protein levels, mitochondrial morphology, and function in AML cells. ...RESULTS: TMQ0153 treatment significantly reduced OPA1 and mitofusin (MFN)2 protein levels and disrupted the mitochondrial morphology and …
Effects of TMQ0153 treatment on OPA1 and mitofusin (MFN)2 protein levels, mitochondrial morphology, and function in AML cells. ...RES …
Disrupting mitochondrial dynamics attenuates ferroptosis and chemotoxicity via upregulating NRF2-mediated FSP1 expression.
Ma S, Qin J, Zhang Y, Luan J, Sun N, Hou G, He J, Xiao Y, Zhang W, Gao M. Ma S, et al. Cell Rep. 2025 Sep 23;44(9):116234. doi: 10.1016/j.celrep.2025.116234. Epub 2025 Sep 3. Cell Rep. 2025. PMID: 40906557 Free article.
Disruption of mitochondrial dynamics by impeding the expression of the central players of mitochondrial dynamics control, dynamin-related protein 1 (DRP1) and Mitofusion1/2, or modifying the expression of optic atrophy 1 (OPA1) inhibits ferroptosis. Mechanistically, a defe …
Disruption of mitochondrial dynamics by impeding the expression of the central players of mitochondrial dynamics control, dynamin-related pr …
Insights on the Genetic and Phenotypic Complexities of Optic Neuropathies.
D'Esposito F, Zeppieri M, Cordeiro MF, Capobianco M, Avitabile A, Gagliano G, Musa M, Barboni P, Gagliano C. D'Esposito F, et al. Genes (Basel). 2024 Nov 29;15(12):1559. doi: 10.3390/genes15121559. Genes (Basel). 2024. PMID: 39766826 Free PMC article.
Results: The results indicate that DOA and LHON are mostly associated with the mitochondrial dysfunction resulting from pathogenic variants in nuclear genes, mainly OPA1, and mitochondrial DNA (mtDNA) genes, respectively. Glaucoma, especially its intricate variants, is lin …
Results: The results indicate that DOA and LHON are mostly associated with the mitochondrial dysfunction resulting from pathogenic variants …
Mitochondrial protein synthesis quality control.
Koludarova L, Battersby BJ. Koludarova L, et al. Hum Mol Genet. 2024 May 22;33(R1):R53-R60. doi: 10.1093/hmg/ddae012. Hum Mol Genet. 2024. PMID: 38280230 Free PMC article. Review.
OPA1 and disease-causing mutants perturb mitochondrial nucleoid distribution.
Macuada J, Molina-Riquelme I, Vidal G, Pérez-Bravo N, Vásquez-Trincado C, Aedo G, Lagos D, Yu-Wai-Man P, Horvath R, Rudge TJ, Cartes-Saavedra B, Eisner V. Macuada J, et al. Cell Death Dis. 2024 Nov 30;15(11):870. doi: 10.1038/s41419-024-07165-9. Cell Death Dis. 2024. PMID: 39616197 Free PMC article.
Optic atrophy protein 1 (OPA1) mediates inner mitochondrial membrane (IMM) fusion and cristae organization. Mutations in OPA1 cause autosomal dominant optic atrophy (ADOA), a leading cause of blindness. ...Opa1-null and cells overexpressing ADOA mutants accum …
Optic atrophy protein 1 (OPA1) mediates inner mitochondrial membrane (IMM) fusion and cristae organization. Mutations in OPA1 …
57 results