000129966 001__ 129966
000129966 005__ 20241125101135.0
000129966 0247_ $$2doi$$a10.1016/j.freeradbiomed.2023.12.006
000129966 0248_ $$2sideral$$a136221
000129966 037__ $$aART-2023-136221
000129966 041__ $$aeng
000129966 100__ $$aGaudó, Paula
000129966 245__ $$aATAD3C regulates ATAD3A assembly and function in the mitochondrial membrane
000129966 260__ $$c2023
000129966 5060_ $$aAccess copy available to the general public$$fUnrestricted
000129966 5203_ $$aMitochondrial ATAD3A is an ATPase Associated with diverse cellular Activities (AAA) domain containing enzyme, involved in the structural organization of the inner mitochondrial membrane and of increasing importance in childhood disease. In humans, two ATAD3A paralogs arose by gene duplication during evolution: ATAD3B and ATAD3C. Here we investigate the cellular activities of the ATAD3C paralog that has been considered a pseudogene. We detected unique ATAD3C peptides in HEK 293T cells, with expression similar to that in human tissues, and showed that it is an integral membrane protein that exposes its carboxy-terminus to the intermembrane space. Overexpression of ATAD3C, but not of ATAD3A, in fibroblasts caused a decrease in cell proliferation and oxygen consumption rate, and an increase of cellular ROS. This was due to the incorporation of ATAD3C monomers in ATAD3A complex in the mitochondrial membrane reducing its size. Consistent with a negative regulation of ATAD3A function in mitochondrial membrane organization, ATAD3C expression led to increased accumulation of respiratory chain dimeric CIII in the inner membrane, to the detriment to that assembled in respiratory supercomplexes. Our results demonstrate a negative dominant role of the ATAD3C paralog with implications for mitochondrial OXPHOS function and suggest that its expression regulates ATAD3A in the cell.
000129966 536__ $$9info:eu-repo/grantAgreement/ES/ISCIII-EDRF/PT17-0019
000129966 540__ $$9info:eu-repo/semantics/openAccess$$aby$$uhttp://creativecommons.org/licenses/by/3.0/es/
000129966 590__ $$a7.1$$b2023
000129966 592__ $$a1.752$$b2023
000129966 591__ $$aENDOCRINOLOGY & METABOLISM$$b15 / 186 = 0.081$$c2023$$dQ1$$eT1
000129966 593__ $$aPhysiology (medical)$$c2023$$dQ1
000129966 591__ $$aBIOCHEMISTRY & MOLECULAR BIOLOGY$$b35 / 313 = 0.112$$c2023$$dQ1$$eT1
000129966 593__ $$aBiochemistry$$c2023$$dQ1
000129966 594__ $$a14.0$$b2023
000129966 655_4 $$ainfo:eu-repo/semantics/article$$vinfo:eu-repo/semantics/publishedVersion
000129966 700__ $$ade Tomás-Mateo, Elena
000129966 700__ $$0(orcid)0000-0003-0145-3020$$aGarrido-Pérez, Nuria$$uUniversidad de Zaragoza
000129966 700__ $$aSantana, Alfredo
000129966 700__ $$0(orcid)0000-0002-0269-7337$$aRuiz-Pesini, Eduardo$$uUniversidad de Zaragoza
000129966 700__ $$0(orcid)0000-0003-1770-6299$$aMontoya, Julio$$uUniversidad de Zaragoza
000129966 700__ $$0(orcid)0000-0002-8585-6371$$aBayona-Bafaluy, Pilar$$uUniversidad de Zaragoza
000129966 7102_ $$11002$$2060$$aUniversidad de Zaragoza$$bDpto. Bioq.Biolog.Mol. Celular$$cÁrea Bioquímica y Biolog.Mole.
000129966 773__ $$g211 (2023), 114-126$$pFree radic. biol. med.$$tFREE RADICAL BIOLOGY AND MEDICINE$$x0891-5849
000129966 8564_ $$s6378689$$uhttps://zaguan.unizar.es/record/129966/files/texto_completo.pdf$$yVersión publicada
000129966 8564_ $$s2466720$$uhttps://zaguan.unizar.es/record/129966/files/texto_completo.jpg?subformat=icon$$xicon$$yVersión publicada
000129966 909CO $$ooai:zaguan.unizar.es:129966$$particulos$$pdriver
000129966 951__ $$a2024-11-22-12:00:36
000129966 980__ $$aARTICLE