<?xml version="1.0" encoding="ISO-8859-1"?><article xmlns:mml="http://www.w3.org/1998/Math/MathML" xmlns:xlink="http://www.w3.org/1999/xlink" xmlns:xsi="http://www.w3.org/2001/XMLSchema-instance">
<front>
<journal-meta>
<journal-id>1405-888X</journal-id>
<journal-title><![CDATA[TIP. Revista especializada en ciencias químico-biológicas]]></journal-title>
<abbrev-journal-title><![CDATA[TIP]]></abbrev-journal-title>
<issn>1405-888X</issn>
<publisher>
<publisher-name><![CDATA[Universidad Nacional Autónoma de México, Facultad de Estudios Superiores Zaragoza]]></publisher-name>
</publisher>
</journal-meta>
<article-meta>
<article-id>S1405-888X2021000100214</article-id>
<article-id pub-id-type="doi">10.22201/fesz.23958723e.2021.370</article-id>
<title-group>
<article-title xml:lang="es"><![CDATA[Viaje al centro de la mitocondria: importación de proteínas, sus alteraciones y enfermedades relacionadas]]></article-title>
<article-title xml:lang="en"><![CDATA[Journey to the center of mitochondria: Protein import, its alterations and related diseases]]></article-title>
</title-group>
<contrib-group>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Avendaño-Monsalve]]></surname>
<given-names><![CDATA[Maria Clara]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Ponce-Rojas]]></surname>
<given-names><![CDATA[José Carlos]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Funes]]></surname>
<given-names><![CDATA[Soledad]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
</contrib-group>
<aff id="Af1">
<institution><![CDATA[,Universidad Nacional Autónoma de México Instituto de Fisiología Celular Departamento de Genética Molecular]]></institution>
<addr-line><![CDATA[ Ciudad de México]]></addr-line>
<country>Mexico</country>
</aff>
<aff id="Af2">
<institution><![CDATA[,University of California Departamento de Biología Molecular, Celular y del Desarrollo ]]></institution>
<addr-line><![CDATA[ ]]></addr-line>
<country>USA</country>
</aff>
<pub-date pub-type="pub">
<day>00</day>
<month>00</month>
<year>2021</year>
</pub-date>
<pub-date pub-type="epub">
<day>00</day>
<month>00</month>
<year>2021</year>
</pub-date>
<volume>24</volume>
<copyright-statement/>
<copyright-year/>
<self-uri xlink:href="http://www.scielo.org.mx/scielo.php?script=sci_arttext&amp;pid=S1405-888X2021000100214&amp;lng=en&amp;nrm=iso"></self-uri><self-uri xlink:href="http://www.scielo.org.mx/scielo.php?script=sci_abstract&amp;pid=S1405-888X2021000100214&amp;lng=en&amp;nrm=iso"></self-uri><self-uri xlink:href="http://www.scielo.org.mx/scielo.php?script=sci_pdf&amp;pid=S1405-888X2021000100214&amp;lng=en&amp;nrm=iso"></self-uri><abstract abstract-type="short" xml:lang="es"><p><![CDATA[Resumen Las mitocondrias son organelos fascinantes, no solo porque son el sitio en donde se genera la energía de las células, sino por su relevancia en procesos y patologías de interés médico. La gran mayoría de las proteínas que constituyen el proteoma mitocondrial están codificadas en el núcleo y se traducen por ribosomas citosólicos, por lo que deben ser identificadas correctamente para ser distribuidas e insertadas en cada uno de los subcompartimentos mitocondriales. En este artículo realizamos una descripción de las maquinarias de importación mitocondrial, además de las diferentes respuestas celulares que contrarrestan las alteraciones en los procesos de transporte de las proteínas o cuando existe una disfunción mitocondrial. Finalmente, mencionamos las enfermedades causadas por mutaciones en los complejos transportadores y de distribución de las proteínas de este organelo, que se han identificado hasta la fecha.]]></p></abstract>
<abstract abstract-type="short" xml:lang="en"><p><![CDATA[Abstract Mitochondria are exciting organelles not only because they are in charge of the energy production within the cell, but also because of their relevance in processes and pathologies of medical interest. The vast majority of the proteins that constitute the mitochondrial proteome are encoded in the nucleus and translated by cytosolic ribosomes, hence these have to be correctly identified in order to be distributed and inserted in each of the mitochondrial subcompartments. In this review, we describe the mitochondrial import machineries, as well as the different cellular responses to alterations in protein transport processes or when a mitochondrial dysfunction arises. Lastly, we give an overview of the identified pathologies triggered by mitochondrial dysfunction derived from mutations in the components of the import and sorting machineries of the organelle.]]></p></abstract>
<kwd-group>
<kwd lng="es"><![CDATA[importación]]></kwd>
<kwd lng="es"><![CDATA[presecuencia]]></kwd>
<kwd lng="es"><![CDATA[translocación de proteínas]]></kwd>
<kwd lng="es"><![CDATA[estrés]]></kwd>
<kwd lng="es"><![CDATA[mitocondriopatías]]></kwd>
<kwd lng="en"><![CDATA[protein import]]></kwd>
<kwd lng="en"><![CDATA[presequence]]></kwd>
<kwd lng="en"><![CDATA[protein translocation]]></kwd>
<kwd lng="en"><![CDATA[stress]]></kwd>
<kwd lng="en"><![CDATA[mitochondriopathies]]></kwd>
</kwd-group>
</article-meta>
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