000118629 001__ 118629
000118629 005__ 20230519145536.0
000118629 0247_ $$2doi$$a10.3390/genes12071031
000118629 0248_ $$2sideral$$a126876
000118629 037__ $$aART-2021-126876
000118629 041__ $$aeng
000118629 100__ $$0(orcid)0000-0002-0269-7337$$aRuiz Pesini E.$$uUniversidad de Zaragoza
000118629 245__ $$aMolecular insights into mitochondrial protein translocation and human disease
000118629 260__ $$c2021
000118629 5060_ $$aAccess copy available to the general public$$fUnrestricted
000118629 5203_ $$aIn human mitochondria, mtDNA encodes for only 13 proteins, all components of the OXPHOS system. The rest of the mitochondrial components, which make up approximately 99% of its proteome, are encoded in the nuclear genome, synthesized in cytosolic ribosomes and imported into mitochondria. Different import machineries translocate mitochondrial precursors, depending on their nature and the final destination inside the organelle. The proper and coordinated function of these molecular pathways is critical for mitochondrial homeostasis. Here, we will review molecular details about these pathways, which components have been linked to human disease and future perspectives on the field to expand the genetic landscape of mitochondrial diseases. © 2021 by the authors. Licensee MDPI, Basel, Switzerland.
000118629 540__ $$9info:eu-repo/semantics/openAccess$$aby$$uhttp://creativecommons.org/licenses/by/3.0/es/
000118629 590__ $$a4.141$$b2021
000118629 591__ $$aGENETICS & HEREDITY$$b72 / 177 = 0.407$$c2021$$dQ2$$eT2
000118629 592__ $$a1.032$$b2021
000118629 593__ $$aGenetics (clinical)$$c2021$$dQ2
000118629 593__ $$aGenetics$$c2021$$dQ2
000118629 594__ $$a5.0$$b2021
000118629 655_4 $$ainfo:eu-repo/semantics/article$$vinfo:eu-repo/semantics/publishedVersion
000118629 700__ $$0(orcid)0000-0003-1770-6299$$aMontoya Villarroya J.$$uUniversidad de Zaragoza
000118629 700__ $$0(orcid)0000-0003-2645-3983$$aPacheu Grau D.$$uUniversidad de Zaragoza
000118629 7102_ $$11002$$2060$$aUniversidad de Zaragoza$$bDpto. Bioq.Biolog.Mol. Celular$$cÁrea Bioquímica y Biolog.Mole.
000118629 773__ $$g12, 7 (2021), 1031 [25 pp]$$pGenes (Basel)$$tGenes$$x2073-4425
000118629 8564_ $$s2057718$$uhttps://zaguan.unizar.es/record/118629/files/texto_completo.pdf$$yVersión publicada
000118629 8564_ $$s2673521$$uhttps://zaguan.unizar.es/record/118629/files/texto_completo.jpg?subformat=icon$$xicon$$yVersión publicada
000118629 909CO $$ooai:zaguan.unizar.es:118629$$particulos$$pdriver
000118629 951__ $$a2023-05-18-15:35:43
000118629 980__ $$aARTICLE