118629 20230519145536.0 doi 10.3390/genes12071031 sideral 126876 ART-2021-126876 eng Ruiz Pesini E. Universidad de Zaragoza (orcid)0000-0002-0269-7337 Molecular insights into mitochondrial protein translocation and human disease 2021 Access copy available to the general public Unrestricted In 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. info:eu-repo/semantics/openAccess by http://creativecommons.org/licenses/by/3.0/es/ 4.141 2021 GENETICS & HEREDITY 72 / 177 = 0.407 2021 Q2 T2 1.032 2021 Genetics (clinical) 2021 Q2 Genetics 2021 Q2 5.0 2021 info:eu-repo/semantics/article info:eu-repo/semantics/publishedVersion Montoya Villarroya J. Universidad de Zaragoza (orcid)0000-0003-1770-6299 Pacheu Grau D. Universidad de Zaragoza (orcid)0000-0003-2645-3983 1002 060 Universidad de Zaragoza Dpto. Bioq.Biolog.Mol. Celular Área Bioquímica y Biolog.Mole. 12, 7 (2021), 1031 [25 pp] Genes (Basel) Genes 2073-4425 2057718 http://zaguan.unizar.es/record/118629/files/texto_completo.pdf Versión publicada 2673521 http://zaguan.unizar.es/record/118629/files/texto_completo.jpg?subformat=icon icon Versión publicada oai:zaguan.unizar.es:118629 articulos driver 2023-05-18-15:35:43 ARTICLE