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Molecular insights into mitochondrial protein translocation and human disease
Authors
Montoya Villarroya J.
Pacheu Grau D.
Ruiz Pesini E.
Publication date
1 January 2021
Publisher
'MDPI AG'
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Abstract
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
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Repositorio Universidad de Zaragoza
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oai:zaguan.unizar.es:118629
Last time updated on 12/10/2022