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(PDF) Dinámica mitocondrial en las enfermedades neurodegenerativas

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caracterizado por la disminución gradual, progresiva y selectiva de las funciones del sistema nervioso. La etiología de estas patologías aún se desconoce, sin embargo, se ha propuesto que la función mitocondrial pudiese estar participando en el establecimiento de estas enfermedades, debido al alto requerimiento energético que tienen las neuronas para realizar sus funciones fisiológicas. La mitocondria es un organelo dinámico que puede cambiar su morfología y función en respuesta a diferentes estímulos fisiológicos, por ello se ha empezado a estudiar a la dinámica mitocondrial como uno de los principales reguladores de la supervivencia celular. Este evento comprende diferentes procesos como la generación de nuevas mitocondrias y su eliminación cuando ya no son funcionales, así como los procesos de fusión y fisión mitocondrial y el tráfico de estos organelos en el entorno celular. Todos estos procesos son altamente regulados y tienen como finalidad la óptima funcionalidad de la mitocondria y la homeostasis celular.","publication_name":"Gaceta de M�xico","grobid_abstract_attachment_id":"77644669"},"document_type":"paper","pre_hit_view_count_baseline":null,"quality":"high","language":"es","title":"Dinámica mitocondrial en las enfermedades neurodegenerativas","broadcastable":true,"draft":null,"has_indexable_attachment":true,"indexable":true}}["work"]; window.loswp.workCoauthors = [44799785]; window.loswp.locale = "en"; window.loswp.countryCode = "SG"; window.loswp.cwvAbTestBucket = ""; window.loswp.designVariant = "ds_vanilla"; window.loswp.fullPageMobileSutdModalVariant = "full_page_mobile_sutd_modal"; window.loswp.useOptimizedScribd4genScript = false; window.loginModal = {}; window.loginModal.appleClientId = 'edu.academia.applesignon';</script><script defer="" src="https://accounts.google.com/gsi/client"></script><div class="ds-loswp-container"><div class="ds-work-card--grid-container"><div class="ds-work-card--container js-loswp-work-card"><div class="ds-work-card--cover"><div class="ds-work-cover--wrapper"><div class="ds-work-cover--container"><button class="ds-work-cover--clickable js-swp-download-button" data-signup-modal="{&quot;location&quot;:&quot;swp-splash-paper-cover&quot;,&quot;attachmentId&quot;:77644669,&quot;attachmentType&quot;:&quot;pdf&quot;}"><img alt="First page of “Dinámica mitocondrial en las enfermedades neurodegenerativas”" class="ds-work-cover--cover-thumbnail" src="https://0.academia-photos.com/attachment_thumbnails/77644669/mini_magick20211229-31892-ehyqpl.png?1640830456" /><img alt="PDF Icon" class="ds-work-cover--file-icon" src="//a.academia-assets.com/images/single_work_splash/adobe_icon.svg" /><div class="ds-work-cover--hover-container"><span class="material-symbols-outlined" style="font-size: 20px" translate="no">download</span><p>Download Free PDF</p></div><div class="ds-work-cover--ribbon-container">Download Free PDF</div><div class="ds-work-cover--ribbon-triangle"></div></button></div></div></div><div class="ds-work-card--work-information"><h1 class="ds-work-card--work-title">Dinámica mitocondrial en las enfermedades neurodegenerativas</h1><div class="ds-work-card--work-authors ds-work-card--detail"><a class="ds-work-card--author js-wsj-grid-card-author ds2-5-body-md ds2-5-body-link" data-author-id="44799785" href="https://independent.academia.edu/MinaK%C3%B6nigsberg"><img alt="Profile image of Mina Königsberg" class="ds-work-card--author-avatar" src="//a.academia-assets.com/images/s65_no_pic.png" />Mina Königsberg</a></div><div class="ds-work-card--detail"><p class="ds-work-card--detail ds2-5-body-sm">Gaceta de M�xico</p><div class="ds-work-card--work-metadata"><div class="ds-work-card--work-metadata__stat"><span class="material-symbols-outlined" style="font-size: 20px" translate="no">visibility</span><p class="ds2-5-body-sm" id="work-metadata-view-count">…</p></div><div class="ds-work-card--work-metadata__stat"><span class="material-symbols-outlined" style="font-size: 20px" translate="no">description</span><p class="ds2-5-body-sm">8 pages</p></div><div class="ds-work-card--work-metadata__stat"><span class="material-symbols-outlined" style="font-size: 20px" translate="no">link</span><p class="ds2-5-body-sm">1 file</p></div></div><script>(async () => { const workId = 66463843; 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La etiología de estas patologías aún se desconoce, sin embargo, se ha propuesto que la función mitocondrial pudiese estar participando en el establecimiento de estas enfermedades, debido al alto requerimiento energético que tienen las neuronas para realizar sus funciones fisiológicas. La mitocondria es un organelo dinámico que puede cambiar su morfología y función en respuesta a diferentes estímulos fisiológicos, por ello se ha empezado a estudiar a la dinámica mitocondrial como uno de los principales reguladores de la supervivencia celular. Este evento comprende diferentes procesos como la generación de nuevas mitocondrias y su eliminación cuando ya no son funcionales, así como los procesos de fusión y fisión mitocondrial y el tráfico de estos organelos en el entorno celular. Todos estos procesos son altamente regulados y tienen como finalidad la óptima funcionalidad de la mitocondria y la homeostasis celular.</p><div class="ds-work-card--button-container"><button class="ds2-5-button js-swp-download-button" data-signup-modal="{&quot;location&quot;:&quot;continue-reading-button--work-card&quot;,&quot;attachmentId&quot;:77644669,&quot;attachmentType&quot;:&quot;pdf&quot;,&quot;workUrl&quot;:&quot;https://www.academia.edu/66463843/Din%C3%A1mica_mitocondrial_en_las_enfermedades_neurodegenerativas&quot;}">See full PDF</button><button class="ds2-5-button ds2-5-button--secondary js-swp-download-button" data-signup-modal="{&quot;location&quot;:&quot;download-pdf-button--work-card&quot;,&quot;attachmentId&quot;:77644669,&quot;attachmentType&quot;:&quot;pdf&quot;,&quot;workUrl&quot;:&quot;https://www.academia.edu/66463843/Din%C3%A1mica_mitocondrial_en_las_enfermedades_neurodegenerativas&quot;}"><span class="material-symbols-outlined" style="font-size: 20px" translate="no">download</span>Download PDF</button></div><div class="ds-signup-banner-trigger-container"><div class="ds-signup-banner-trigger"></div></div><div class="ds-signup-banner"><div id="ds-signup-banner-close-button"><button class="ds2-5-button ds2-5-button--secondary ds2-5-button--inverse"><span class="material-symbols-outlined" style="font-size: 20px" translate="no">close</span></button></div><div class="ds-signup-banner-ctas"><img src="//a.academia-assets.com/images/academia-logo-capital-white.svg" /><h4 class="ds2-5-heading-serif-sm">Sign up for access to the world's latest research.</h4><button class="ds2-5-button ds2-5-button--inverse ds2-5-button--full-width js-swp-download-button" data-signup-modal="{&quot;location&quot;:&quot;signup-banner&quot;}">Sign up for free</button></div><div class="ds-signup-banner-divider"></div><div class="ds-signup-banner-reasons"><div class="ds-signup-banner-reasons-item"><span class="material-symbols-outlined" style="font-size: 24px" translate="no">check</span><span>Get notified about relevant papers</span></div><div class="ds-signup-banner-reasons-item"><span class="material-symbols-outlined" style="font-size: 24px" translate="no">check</span><span>Save papers to use in your research</span></div><div class="ds-signup-banner-reasons-item"><span class="material-symbols-outlined" style="font-size: 24px" translate="no">check</span><span>Join the discussion with peers</span></div><div class="ds-signup-banner-reasons-item"><span class="material-symbols-outlined" style="font-size: 24px" translate="no">check</span><span>Track your impact</span></div></div></div><script>(() => { // Set up signup banner show/hide behavior: // 1. 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In recent years the study of the role of the mitochondria in ageing and disease pathophysiology has increased providing new knowledge in animal models as well as in human beings. Objective: in this review we present a brief descritption of the mitocondria, its DNA and its role in ageing and in particular in neurodegenrative diseases. Method: along the review we cover the mitochondrial function and dysfunction in neurodegenerative diseases such as Huntington, Parkinson, Alzheimer and amyotrophic lateral sclerosis emphasizing on recent advances. Conclusion: the mitochondria plays a relevant role in the pathogenesis of various neurodegenerative diseases, the understanding of the cellular mechanisms involved would allow the development of new therapeutic interventions.</p><div class="ds-related-work--ctas"><button class="ds2-5-text-link ds2-5-text-link--inline js-swp-download-button" data-signup-modal="{&quot;location&quot;:&quot;wsj-grid-card-download-pdf-modal&quot;,&quot;work_title&quot;:&quot;Papel de la función mitocondrial en las enfermedades neurodegenerativas&quot;,&quot;attachmentId&quot;:92332032,&quot;attachmentType&quot;:&quot;pdf&quot;,&quot;work_url&quot;:&quot;https://www.academia.edu/88340725/Papel_de_la_funci%C3%B3n_mitocondrial_en_las_enfermedades_neurodegenerativas&quot;,&quot;alternativeTracking&quot;:true}"><span class="material-symbols-outlined" style="font-size: 18px" translate="no">download</span><span class="ds2-5-text-link__content">Download free PDF</span></button><a class="ds2-5-text-link ds2-5-text-link--inline js-wsj-grid-card-view-pdf" href="https://www.academia.edu/88340725/Papel_de_la_funci%C3%B3n_mitocondrial_en_las_enfermedades_neurodegenerativas"><span class="ds2-5-text-link__content">View PDF</span><span class="material-symbols-outlined" style="font-size: 18px" translate="no">chevron_right</span></a></div></div><div class="ds-related-work--container js-wsj-grid-card" data-collection-position="1" data-entity-id="7114969" data-sort-order="default"><a class="ds-related-work--title js-wsj-grid-card-title ds2-5-body-md ds2-5-body-link" href="https://www.academia.edu/7114969/Din%C3%A1mica_mitocondrial_un_potencial_nuevo_blanco_terap%C3%A9utico_para_la_insuficiencia_cardiaca">Dinámica mitocondrial: un potencial nuevo blanco terapéutico para la insuficiencia cardiaca</a><div class="ds-related-work--metadata"><a class="js-wsj-grid-card-author ds2-5-body-sm ds2-5-body-link" data-author-id="12245069" href="https://uv-cl.academia.edu/CamilaValentina">Camila Valentina</a></div><p class="ds-related-work--metadata ds2-5-body-xs">Revista Espanola De Cardiologia, 2011</p><p class="ds-related-work--abstract ds2-5-body-sm">La insuficiencia cardiaca es una afecció n compleja caracterizada por la incapacidad del corazó n para responder con un gasto cardiaco acorde a la demanda metabó lica del resto de los tejidos. Esta enfermedad representa el estado terminal de las cardiopatías isqué mica, valvular o hipertensiva 1 . En los países industrializados, donde la esperanza de vida de la població n y la sobrevida a otras enfermedades cardiacas es elevada, la insuficiencia cardiaca es una enfermedad de alta prevalencia 2,3 . La comprensió n de la fisiopatología de la insuficiencia cardiaca ha permitido el establecimiento de la terapia está ndar basada en el uso de bloqueadores beta combinado con el bloqueo del sistema renina-angiotensina-aldosterona a travé s de inhibidores de la enzima de conversió n de la angiotensina, antagonistas del receptor de la angiotensina II, antagonistas del receptor de aldosterona y, en algunos casos, la terapia de resincronizació n 4 . A pesar de estos avances, la mortalidad anual asociada a insuficiencia cardiaca se mantiene cercana al 10%, y se desconoce la razó n por la cual esta enfermedad progresa incluso con una terapia ó ptima 5 . Estos antecedentes ponen en evidencia la necesidad de desarrollar nuevos blancos terapé uticos dirigidos a otros mecanismos moleculares y celulares alterados en esta enfermedad.</p><div class="ds-related-work--ctas"><button class="ds2-5-text-link ds2-5-text-link--inline js-swp-download-button" data-signup-modal="{&quot;location&quot;:&quot;wsj-grid-card-download-pdf-modal&quot;,&quot;work_title&quot;:&quot;Dinámica mitocondrial: un potencial nuevo blanco terapéutico para la insuficiencia cardiaca&quot;,&quot;attachmentId&quot;:48595627,&quot;attachmentType&quot;:&quot;pdf&quot;,&quot;work_url&quot;:&quot;https://www.academia.edu/7114969/Din%C3%A1mica_mitocondrial_un_potencial_nuevo_blanco_terap%C3%A9utico_para_la_insuficiencia_cardiaca&quot;,&quot;alternativeTracking&quot;:true}"><span class="material-symbols-outlined" style="font-size: 18px" translate="no">download</span><span class="ds2-5-text-link__content">Download free PDF</span></button><a class="ds2-5-text-link ds2-5-text-link--inline js-wsj-grid-card-view-pdf" href="https://www.academia.edu/7114969/Din%C3%A1mica_mitocondrial_un_potencial_nuevo_blanco_terap%C3%A9utico_para_la_insuficiencia_cardiaca"><span class="ds2-5-text-link__content">View PDF</span><span class="material-symbols-outlined" style="font-size: 18px" translate="no">chevron_right</span></a></div></div><div class="ds-related-work--container js-wsj-grid-card" data-collection-position="2" data-entity-id="50543550" data-sort-order="default"><a class="ds-related-work--title js-wsj-grid-card-title ds2-5-body-md ds2-5-body-link" href="https://www.academia.edu/50543550/Rol_del_eje_tirotr%C3%B3pico_en_enfermedades_neurodegenerativas">Rol del eje tirotrópico en enfermedades neurodegenerativas</a><div class="ds-related-work--metadata"><a class="js-wsj-grid-card-author ds2-5-body-sm ds2-5-body-link" data-author-id="42135990" href="https://independent.academia.edu/LuisAlmaguerMederos">Luis Almaguer-Mederos</a></div><p class="ds-related-work--abstract ds2-5-body-sm">Las hormonas tiroideas actúan como reguladores primarios del metabolismo intermediario en todos los tejidos del organismo; tienen funciones de gran trascendencia a nivel del sistema nervioso, donde ejercen acciones potencialmente neuroprotectoras. Los niveles de las hormonas tiroideas están sujetos a una estrecha regulación en el sistema nervioso y se sugiere que incluso ligeras desviaciones del rango de normalidad pueden estar asociadas a enfermedades neurodegenerativas. En esta revisión se resumieron los hallazgos existentes en torno al papel de las hormonas tiroideas en la fisiología normal y patológica del sistema nervioso, con énfasis en enfermedades neurodegenerativas y los mecanismos moleculares implicados. Las evidencias indican un rol significativo para las hormonas tiroideas en varias enfermedades neurodegenerativas, si bien es necesaria la realización de estudios prospectivos de mayor envergadura para precisar su función y evaluar su utilidad como bio-marcadores y dianas ...</p><div class="ds-related-work--ctas"><button class="ds2-5-text-link ds2-5-text-link--inline js-swp-download-button" data-signup-modal="{&quot;location&quot;:&quot;wsj-grid-card-download-pdf-modal&quot;,&quot;work_title&quot;:&quot;Rol del eje tirotrópico en enfermedades neurodegenerativas&quot;,&quot;attachmentId&quot;:68484987,&quot;attachmentType&quot;:&quot;pdf&quot;,&quot;work_url&quot;:&quot;https://www.academia.edu/50543550/Rol_del_eje_tirotr%C3%B3pico_en_enfermedades_neurodegenerativas&quot;,&quot;alternativeTracking&quot;:true}"><span class="material-symbols-outlined" style="font-size: 18px" translate="no">download</span><span class="ds2-5-text-link__content">Download free PDF</span></button><a class="ds2-5-text-link ds2-5-text-link--inline js-wsj-grid-card-view-pdf" href="https://www.academia.edu/50543550/Rol_del_eje_tirotr%C3%B3pico_en_enfermedades_neurodegenerativas"><span class="ds2-5-text-link__content">View PDF</span><span class="material-symbols-outlined" style="font-size: 18px" translate="no">chevron_right</span></a></div></div><div class="ds-related-work--container js-wsj-grid-card" data-collection-position="3" data-entity-id="33327157" data-sort-order="default"><a class="ds-related-work--title js-wsj-grid-card-title ds2-5-body-md ds2-5-body-link" href="https://www.academia.edu/33327157/Las_mitocondrias_como_blanco_terap%C3%A9utico">Las mitocondrias como blanco terapéutico</a><div class="ds-related-work--metadata"><a class="js-wsj-grid-card-author ds2-5-body-sm ds2-5-body-link" data-author-id="65137346" href="https://independent.academia.edu/LatourManuel">Manuel Latour</a></div><p class="ds-related-work--abstract ds2-5-body-sm">Este artículo puede ser consultado en versión completa en RESUMEN Las mitocondrias tienen un importante papel en la producción de energía en las células. Además de sus bien conocidos papeles en la fosforilación oxidativa y en el metabolismo, ahora es claro que las mitocondrias son centrales en la muerte celular, neoplasias, en la diferenciación celular, en el sistema inmune innato, en la detección de los niveles de oxígeno y de la hipoxia y en el meta-bolismo del calcio. Estos organelos están siendo progresivamente reconocidos como elementos clave en muchas áreas de la ciencia biomédica. La alteración de cualquiera de los papeles funcionales de las mitocondrias contribuye a diversas patologías, por lo que hace de este organelo un blanco terapéutico potencialmente importante, aunque no considerado así hasta recientemente. La disfunción mitocondrial está a menudo asociada con el daño oxidativo, la alteración en la homeostasis de calcio, defectos en la síntesis de ATP o con la inducción del poro de la transición de la permeabilidad. Consecuentemente, las terapias diseñadas para prevenir este tipo de daños son benéfi cas en diversos padecimientos que incluso podrían parecer no relacionados. En esta revisión resaltamos las propiedades biológicas que hacen a la mitocondria elemento determinante de la salud y de la enfermedad. Además se describen las po-sibles estrategias farmacológicas que están siendo desarrolladas e incluso, algunas que ya se aplican en los seres humanos para corregir la disfunción mitocondrial. La mitocondria considerada como un blanco terapéutico junto con los compuestos que ya se usan para corregir su disfunción daría lugar a lo que llamaríamos farmacología mitocondrial, la cual es una disciplina muy prometedora con un enorme potencial para nuevos abordajes terapéuticos con implicaciones en múltiples patologías. SUMMARY Mitochondria have an important role in energy production in cells. In addition to their well known roles in oxidative phosphorylation and metabolism, it is now clear that the mitochondria are also central to cell death, neoplasia, cell differentiation, and the innate immune system, for detecting levels of oxygen and hypoxia and calcium homeostasis. These organelles are increasingly being recognized as key elements in many areas of biomedical science. The disruption of any of the functional roles of mitochondria contributes to diseases, so this organelle turns on a potentially important therapeutic target apparently not considered until recently. Mitochondrial dysfunction is often associated with oxidative damage, alterations in calcium homeostasis and defects in the synthesis of ATP or induction of the permeability transition pore. Thus, therapies designed to prevent this type of damage are benefi cial in various conditions that may even seem unrelated. In this review we highlight the biological properties that make mitochondria determinants of health and disease. Besides the possible pharmacological strategies www.medigraphic.org.mx</p><div class="ds-related-work--ctas"><button class="ds2-5-text-link ds2-5-text-link--inline js-swp-download-button" data-signup-modal="{&quot;location&quot;:&quot;wsj-grid-card-download-pdf-modal&quot;,&quot;work_title&quot;:&quot;Las mitocondrias como blanco terapéutico&quot;,&quot;attachmentId&quot;:53388390,&quot;attachmentType&quot;:&quot;pdf&quot;,&quot;work_url&quot;:&quot;https://www.academia.edu/33327157/Las_mitocondrias_como_blanco_terap%C3%A9utico&quot;,&quot;alternativeTracking&quot;:true}"><span class="material-symbols-outlined" style="font-size: 18px" translate="no">download</span><span class="ds2-5-text-link__content">Download free PDF</span></button><a class="ds2-5-text-link ds2-5-text-link--inline js-wsj-grid-card-view-pdf" href="https://www.academia.edu/33327157/Las_mitocondrias_como_blanco_terap%C3%A9utico"><span class="ds2-5-text-link__content">View PDF</span><span class="material-symbols-outlined" style="font-size: 18px" translate="no">chevron_right</span></a></div></div><div class="ds-related-work--container js-wsj-grid-card" data-collection-position="4" data-entity-id="23151840" data-sort-order="default"><a class="ds-related-work--title js-wsj-grid-card-title ds2-5-body-md ds2-5-body-link" href="https://www.academia.edu/23151840/Dianas_farmacol%C3%B3gicas_en_las_enfermedades_neurodegenerativas">Dianas farmacológicas en las enfermedades neurodegenerativas</a><div class="ds-related-work--metadata"><a class="js-wsj-grid-card-author ds2-5-body-sm ds2-5-body-link" data-author-id="44929893" href="https://independent.academia.edu/Tom%C3%A1sSegura">Tomás Segura</a></div><div class="ds-related-work--ctas"><button class="ds2-5-text-link ds2-5-text-link--inline js-swp-download-button" data-signup-modal="{&quot;location&quot;:&quot;wsj-grid-card-download-pdf-modal&quot;,&quot;work_title&quot;:&quot;Dianas farmacológicas en las enfermedades neurodegenerativas&quot;,&quot;attachmentId&quot;:43638088,&quot;attachmentType&quot;:&quot;pdf&quot;,&quot;work_url&quot;:&quot;https://www.academia.edu/23151840/Dianas_farmacol%C3%B3gicas_en_las_enfermedades_neurodegenerativas&quot;,&quot;alternativeTracking&quot;:true}"><span class="material-symbols-outlined" style="font-size: 18px" translate="no">download</span><span class="ds2-5-text-link__content">Download free PDF</span></button><a class="ds2-5-text-link ds2-5-text-link--inline js-wsj-grid-card-view-pdf" href="https://www.academia.edu/23151840/Dianas_farmacol%C3%B3gicas_en_las_enfermedades_neurodegenerativas"><span class="ds2-5-text-link__content">View PDF</span><span class="material-symbols-outlined" style="font-size: 18px" translate="no">chevron_right</span></a></div></div><div class="ds-related-work--container js-wsj-grid-card" data-collection-position="5" data-entity-id="65478250" data-sort-order="default"><a class="ds-related-work--title js-wsj-grid-card-title ds2-5-body-md ds2-5-body-link" href="https://www.academia.edu/65478250/Neuropatolog%C3%ADa_De_Las_Demencias_Neurodegenerativas">Neuropatología De Las Demencias Neurodegenerativas</a><div class="ds-related-work--metadata"><a class="js-wsj-grid-card-author ds2-5-body-sm ds2-5-body-link" data-author-id="94856961" href="https://independent.academia.edu/ArriagadaPaulina">Paulina Arriagada</a></div><p class="ds-related-work--metadata ds2-5-body-xs">Revista Médica Clínica Las Condes, 2016</p><div class="ds-related-work--ctas"><button class="ds2-5-text-link ds2-5-text-link--inline js-swp-download-button" data-signup-modal="{&quot;location&quot;:&quot;wsj-grid-card-download-pdf-modal&quot;,&quot;work_title&quot;:&quot;Neuropatología De Las Demencias 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href="https://www.academia.edu/15317352/Glicaci%C3%B3n_de_prote%C3%ADnas_mitocondriales_estr%C3%A9s_oxidativo_y_envejecimiento">Glicación de proteínas mitocondriales, estrés oxidativo y envejecimiento</a><div class="ds-related-work--metadata"><a class="js-wsj-grid-card-author ds2-5-body-sm ds2-5-body-link" data-author-id="34424312" href="https://udl.academia.edu/ReinaldPamplona">Reinald Pamplona</a></div><p class="ds-related-work--metadata ds2-5-body-xs">Revista Española de Geriatría y Gerontología, 2010</p><p class="ds-related-work--abstract ds2-5-body-sm">El anciano en situación crítica: nuevos retos en la asistencia geriátrica del futuro A. López-Soto y E. Sacanella</p><div class="ds-related-work--ctas"><button class="ds2-5-text-link ds2-5-text-link--inline js-swp-download-button" data-signup-modal="{&quot;location&quot;:&quot;wsj-grid-card-download-pdf-modal&quot;,&quot;work_title&quot;:&quot;Glicación de proteínas mitocondriales, estrés oxidativo y envejecimiento&quot;,&quot;attachmentId&quot;:43338953,&quot;attachmentType&quot;:&quot;pdf&quot;,&quot;work_url&quot;:&quot;https://www.academia.edu/15317352/Glicaci%C3%B3n_de_prote%C3%ADnas_mitocondriales_estr%C3%A9s_oxidativo_y_envejecimiento&quot;,&quot;alternativeTracking&quot;:true}"><span class="material-symbols-outlined" style="font-size: 18px" translate="no">download</span><span class="ds2-5-text-link__content">Download free PDF</span></button><a class="ds2-5-text-link ds2-5-text-link--inline js-wsj-grid-card-view-pdf" href="https://www.academia.edu/15317352/Glicaci%C3%B3n_de_prote%C3%ADnas_mitocondriales_estr%C3%A9s_oxidativo_y_envejecimiento"><span class="ds2-5-text-link__content">View PDF</span><span class="material-symbols-outlined" style="font-size: 18px" translate="no">chevron_right</span></a></div></div><div class="ds-related-work--container js-wsj-grid-card" data-collection-position="7" data-entity-id="33327154" data-sort-order="default"><a class="ds-related-work--title js-wsj-grid-card-title ds2-5-body-md ds2-5-body-link" href="https://www.academia.edu/33327154/Neurolog%C3%ADa_Argentina_Revisi%C3%B3n_Tres_preguntas_y_una_respuesta_algoritmo_diagn%C3%B3stico_molecular_en_enfermedades_mitocondriales">Neurología Argentina Revisión Tres preguntas y una respuesta: algoritmo diagnóstico molecular en enfermedades mitocondriales</a><div class="ds-related-work--metadata"><a class="js-wsj-grid-card-author ds2-5-body-sm ds2-5-body-link" data-author-id="65137346" href="https://independent.academia.edu/LatourManuel">Manuel Latour</a></div><p class="ds-related-work--abstract ds2-5-body-sm">información del artículo Historia del artículo: Recibido el 5 de septiembre de 2012 Aceptado el 6 de octubre de 2012 On-line el 30 de noviembre de 2012 Palabras clave: Trastornos mitocondriales Genética mitocondrial Defectos de la fosforilación oxidativa r e s u m e n Introducción: Las enfermedades mitocondriales representan un grupo de trastornos multisistémicos, que frecuentemente incluyen disfunción del sistema nervioso central y periférico entre sus manifestaciones, causados por diversas mutaciones localizadas tanto en el genoma mitocondrial como en el nuclear. La amplia variabilidad observada en su expresión clínica y la amplitud etiopatogénica complica su diagnóstico molecular.</p><div class="ds-related-work--ctas"><button class="ds2-5-text-link ds2-5-text-link--inline js-swp-download-button" data-signup-modal="{&quot;location&quot;:&quot;wsj-grid-card-download-pdf-modal&quot;,&quot;work_title&quot;:&quot;Neurología Argentina Revisión Tres preguntas y una respuesta: algoritmo diagnóstico molecular en enfermedades mitocondriales&quot;,&quot;attachmentId&quot;:53388389,&quot;attachmentType&quot;:&quot;pdf&quot;,&quot;work_url&quot;:&quot;https://www.academia.edu/33327154/Neurolog%C3%ADa_Argentina_Revisi%C3%B3n_Tres_preguntas_y_una_respuesta_algoritmo_diagn%C3%B3stico_molecular_en_enfermedades_mitocondriales&quot;,&quot;alternativeTracking&quot;:true}"><span class="material-symbols-outlined" style="font-size: 18px" translate="no">download</span><span class="ds2-5-text-link__content">Download free PDF</span></button><a class="ds2-5-text-link ds2-5-text-link--inline js-wsj-grid-card-view-pdf" href="https://www.academia.edu/33327154/Neurolog%C3%ADa_Argentina_Revisi%C3%B3n_Tres_preguntas_y_una_respuesta_algoritmo_diagn%C3%B3stico_molecular_en_enfermedades_mitocondriales"><span class="ds2-5-text-link__content">View PDF</span><span class="material-symbols-outlined" style="font-size: 18px" translate="no">chevron_right</span></a></div></div><div class="ds-related-work--container js-wsj-grid-card" data-collection-position="8" data-entity-id="33590505" data-sort-order="default"><a class="ds-related-work--title js-wsj-grid-card-title ds2-5-body-md ds2-5-body-link" href="https://www.academia.edu/33590505/Mecanismos_de_da%C3%B1o_celular_en_enfermedades_neurodegenerativas">Mecanismos de daño celular en enfermedades neurodegenerativas</a><div class="ds-related-work--metadata"><a class="js-wsj-grid-card-author ds2-5-body-sm ds2-5-body-link" data-author-id="53791949" href="https://independent.academia.edu/MGonzalezHita">M. González-Hita</a><span>, </span><a class="js-wsj-grid-card-author ds2-5-body-sm ds2-5-body-link" data-author-id="56828526" href="https://independent.academia.edu/SSanchezEnriquez">Sergio Sanchez Enriquez</a></div><p class="ds-related-work--metadata ds2-5-body-xs">Investigacion En Salud, 2007</p><div class="ds-related-work--ctas"><button class="ds2-5-text-link ds2-5-text-link--inline js-swp-download-button" data-signup-modal="{&quot;location&quot;:&quot;wsj-grid-card-download-pdf-modal&quot;,&quot;work_title&quot;:&quot;Mecanismos de daño celular en enfermedades neurodegenerativas&quot;,&quot;attachmentId&quot;:53611668,&quot;attachmentType&quot;:&quot;pdf&quot;,&quot;work_url&quot;:&quot;https://www.academia.edu/33590505/Mecanismos_de_da%C3%B1o_celular_en_enfermedades_neurodegenerativas&quot;,&quot;alternativeTracking&quot;:true}"><span class="material-symbols-outlined" style="font-size: 18px" translate="no">download</span><span class="ds2-5-text-link__content">Download free PDF</span></button><a class="ds2-5-text-link ds2-5-text-link--inline js-wsj-grid-card-view-pdf" href="https://www.academia.edu/33590505/Mecanismos_de_da%C3%B1o_celular_en_enfermedades_neurodegenerativas"><span class="ds2-5-text-link__content">View PDF</span><span class="material-symbols-outlined" style="font-size: 18px" translate="no">chevron_right</span></a></div></div><div class="ds-related-work--container js-wsj-grid-card" data-collection-position="9" data-entity-id="54181375" data-sort-order="default"><a class="ds-related-work--title js-wsj-grid-card-title ds2-5-body-md ds2-5-body-link" href="https://www.academia.edu/54181375/Enfermedades_gen_ticas_del_ADN_mitocondrial_humano">Enfermedades gen�ticas del ADN mitocondrial humano</a><div class="ds-related-work--metadata"><a class="js-wsj-grid-card-author ds2-5-body-sm ds2-5-body-link" data-author-id="34405003" href="https://independent.academia.edu/JulioMontoya7">Julio Montoya</a></div><p class="ds-related-work--metadata ds2-5-body-xs">Salud Publica Mexico, 2001</p><p class="ds-related-work--abstract ds2-5-body-sm">Mitochondrial diseases are a group of disorders produced by defects in the oxidative phosphorylation system (Oxphos system), the final pathway of the mitochondrial energetic metabolism, resulting in a deficiency of the biosynthesis of ATP. Part of the polypeptide subunits involved in the Oxphos system are codified by the mitochondrial DNA. In the last years, mutations in this genetic system have been described and associated to well defined clinical syndromes. The clinical features of these disorders are very heterogeneous affecting, in most cases, to different organs and tissues and their correct diagnosis require precise clinical, morphological, biochemical and genetic data. The peculiar genetic characteristics of the mitochondrial DNA (maternal inheritance, polyplasmia and mitotic segregation) give to these disorders very distinctive properties.</p><div class="ds-related-work--ctas"><button class="ds2-5-text-link ds2-5-text-link--inline js-swp-download-button" data-signup-modal="{&quot;location&quot;:&quot;wsj-grid-card-download-pdf-modal&quot;,&quot;work_title&quot;:&quot;Enfermedades gen�ticas del ADN mitocondrial humano&quot;,&quot;attachmentId&quot;:70669465,&quot;attachmentType&quot;:&quot;pdf&quot;,&quot;work_url&quot;:&quot;https://www.academia.edu/54181375/Enfermedades_gen_ticas_del_ADN_mitocondrial_humano&quot;,&quot;alternativeTracking&quot;:true}"><span class="material-symbols-outlined" style="font-size: 18px" translate="no">download</span><span class="ds2-5-text-link__content">Download free PDF</span></button><a class="ds2-5-text-link ds2-5-text-link--inline js-wsj-grid-card-view-pdf" href="https://www.academia.edu/54181375/Enfermedades_gen_ticas_del_ADN_mitocondrial_humano"><span class="ds2-5-text-link__content">View PDF</span><span class="material-symbols-outlined" style="font-size: 18px" translate="no">chevron_right</span></a></div></div></div></div><div class="ds-sticky-ctas--wrapper js-loswp-sticky-ctas hidden"><div class="ds-sticky-ctas--grid-container"><div class="ds-sticky-ctas--container"><button class="ds2-5-button js-swp-download-button" data-signup-modal="{&quot;location&quot;:&quot;continue-reading-button--sticky-ctas&quot;,&quot;attachmentId&quot;:77644669,&quot;attachmentType&quot;:&quot;pdf&quot;,&quot;workUrl&quot;:null}">See full PDF</button><button class="ds2-5-button ds2-5-button--secondary js-swp-download-button" data-signup-modal="{&quot;location&quot;:&quot;download-pdf-button--sticky-ctas&quot;,&quot;attachmentId&quot;:77644669,&quot;attachmentType&quot;:&quot;pdf&quot;,&quot;workUrl&quot;:null}"><span class="material-symbols-outlined" style="font-size: 20px" translate="no">download</span>Download PDF</button></div></div></div><div class="ds-below-fold--grid-container"><div class="ds-work--container js-loswp-embedded-document"><div class="attachment_preview" data-attachment="Attachment_77644669" style="display: none"><div class="js-scribd-document-container"><div class="scribd--document-loading js-scribd-document-loader" style="display: block;"><img alt="Loading..." src="//a.academia-assets.com/images/loaders/paper-load.gif" /><p>Loading Preview</p></div></div><div style="text-align: center;"><div class="scribd--no-preview-alert js-preview-unavailable"><p>Sorry, preview is currently unavailable. 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