Investigadors de l\u2019Institut d\u2019Investigaci\u00f3 Biom\u00e8dica de Bellvitge (IDIBELL) han demostrat que un medicament que es fa servir per controlar la diabetis tipus II \u00e9s capa\u00e7 de reparar la medul\u00b7la espinal de models de ratolins adrenoleucodistr\u00f2fia lligada al cromosoma X (ALD-X), una malaltia heredit\u00e0ria que, no tractada, condueix a la par\u00e0lisi, l\u2019estat vegetatiu i la mort.<\/p>\n
Aquest \u00e9s un pas important en el cam\u00ed cap al desenvolupament d’una ter\u00e0pia per a la malaltia en humans ja qu\u00e8 els tractaments actuals s\u00f3n escassos i nom\u00e9s parcialment efica\u00e7os.<\/p>\n
Els resultats d\u2019aquest estudi s\u2019han avan\u00e7at aquest dissabte en l\u2019edici\u00f3 electr\u00f2nica de la revista Brain.<\/p>\n
<\/p>\n
Paper del mitocondri<\/strong><\/p>\n El grup de recerca en malalties neurodegeneratives de l\u2019IDIBELL, liderat per la investigadora ICREA Aurora Pujol, investiga el paper del mitocondri, la central energ\u00e8tica de la c\u00e8l\u00b7lula, en l\u2019adrenoluecodistr\u00f2fia, una malaltia causada per la inactivaci\u00f3 de la prote\u00efna ABCD1, transportadora d’\u00e0cids grassos als peroxisomes, encarregats de degradar productes que la c\u00e8l\u00b7lula ja no necessita. Sense ABCD1 s\u2019acumulen \u00e0cids grassos i radicals lliures en els \u00f2rgans i el plasma sanguini causant la degeneraci\u00f3 de la medul\u00b7la espinal.<\/p>\n El grup de malalties conegudes com leucodistrofies es caracteritza per la p\u00e8rdua progressiva de la beina de mielina, la coberta de greix que actua com un a\u00efllant al voltant de les fibres nervioses. El dany a la beina de mielina impedeix la conducci\u00f3 de les senyals en els nervis afectats i condueix a problemes en l’aparell locomotor.<\/p>\n Fins ara, la implicaci\u00f3 dels mitocondris en la malaltia no era evident. \u201cSab\u00edem per la nostra investigaci\u00f3 recent que l’estr\u00e8s oxidatiu estava implicat. Tamb\u00e9 que la fallida bioenerg\u00e8tica comen\u00e7a abans que apareguin els s\u00edmptomes de la malaltia. Per tant, vam decidir investigar el paper dels mitocondris\u201d, ha explicat la investigadora Aurora Pujol.<\/p>\n <\/p>\n Dany oxidatiu<\/strong><\/p>\n “Vam trobar que els models de ratolins X-ALD mostren una p\u00e8rdua dels mitocondris als 12 mesos d’edat, abans dels s\u00edmptomes de la malaltia, pel que no podria ser una conseq\u00fc\u00e8ncia de la malaltia, sin\u00f3 m\u00e9s aviat un factor que contribueix. Tamb\u00e9 sab\u00edem que la via implicada en la p\u00e8rdua mitocondrial podria ser tractada amb pioglitazona, un medicament que s\u2019utilitza per controlar la diabetis, aix\u00ed que vam decidir provar el seu efecte en els ratolins\u201d ha explicat Pujol.<\/p>\n La pioglitazona atura la degeneraci\u00f3 neural i frena el deteriorament de l’aparell locomotor. Els investigadors van ser capa\u00e7os de demostrar-ho , tant a trav\u00e9s d’an\u00e0lisi de les medul\u00b7les espinals post mortem<\/em>, i tamb\u00e9 in vivo<\/em> fent passar el ratolins d’una s\u00e8rie de proves f\u00edsiques.<\/p>\n Tot i que la ALD-X \u00e9s una malaltia relativament rara amb una incid\u00e8ncia m\u00ednima d’1 en 17 000 homes, hi ha altres trastorns neurodegeneratius causats per la degeneraci\u00f3 de la beina de mielina, com per exemple l\u2019esclerosi m\u00faltiple, i moltes altres en qu\u00e8 tamb\u00e9 estan implicats la fallida bioenerg\u00e8tica, l’estr\u00e8s oxidatiu i la degeneraci\u00f3 dels axons Aix\u00f2 inclou la malaltia de Parkinson, la malaltia de Huntington i la malaltia d’Alzheimer. “\u00c9s possible que els nostres resultats tamb\u00e9 puguin ser rellevants per a aquests desordres\u201d ha dit la investigadora.<\/p>\n “Arran d’aquests prometedors resultats prometedors, amb el professor Patrick Aubourg de l’Hospital Bic\u00eatre, Paris, en breu s\u2019iniciar\u00e0 un assaig cl\u00ednic multic\u00e8ntric de fase II amb pioglitazona en pacients de l’adrenoleucodistr\u00f2fia. Estem contents d’haver fet una contribuci\u00f3 a la recerca d’un tractament simple i efica\u00e7 per a un grup de malalties devastadores\u201d ha concl\u00f2s Pujol.<\/p>\n La investigadora Aurora Pujol ha presentat els resultats d\u2019aquest estudi en la confer\u00e8ncia anual de la Societat Europea de Gen\u00e8tica Humana que se celebra del 8 a l\u201911 de juny a Par\u00eds.<\/p>\n <\/p>\n Refer\u00e8ncia de l\u2019article<\/strong> Investigadors de l\u2019Institut d\u2019Investigaci\u00f3 Biom\u00e8dica de Bellvitge (IDIBELL) han demostrat que un medicament que es fa servir per controlar la diabetis tipus II \u00e9s capa\u00e7 de reparar la medul\u00b7la espinal de models de ratolins adrenoleucodistr\u00f2fia lligada al cromosoma X (ALD-X), una malaltia heredit\u00e0ria que, no tractada, condueix a la par\u00e0lisi, l\u2019estat vegetatiu i la mort. […]<\/p>\n","protected":false},"author":6,"featured_media":19876,"comment_status":"closed","ping_status":"closed","sticky":false,"template":"","format":"standard","meta":{"site-sidebar-layout":"no-sidebar","site-content-layout":"page-builder","ast-site-content-layout":"","site-content-style":"default","site-sidebar-style":"default","ast-global-header-display":"","ast-banner-title-visibility":"","ast-main-header-display":"","ast-hfb-above-header-display":"","ast-hfb-below-header-display":"","ast-hfb-mobile-header-display":"","site-post-title":"","ast-breadcrumbs-content":"","ast-featured-img":"","footer-sml-layout":"","theme-transparent-header-meta":"default","adv-header-id-meta":"","stick-header-meta":"default","header-above-stick-meta":"","header-main-stick-meta":"","header-below-stick-meta":"","astra-migrate-meta-layouts":"default","ast-page-background-enabled":"default","ast-page-background-meta":{"desktop":{"background-color":"","background-image":"","background-repeat":"repeat","background-position":"center center","background-size":"auto","background-attachment":"scroll","background-type":"","background-media":"","overlay-type":"","overlay-color":"","overlay-gradient":""},"tablet":{"background-color":"","background-image":"","background-repeat":"repeat","background-position":"center center","background-size":"auto","background-attachment":"scroll","background-type":"","background-media":"","overlay-type":"","overlay-color":"","overlay-gradient":""},"mobile":{"background-color":"","background-image":"","background-repeat":"repeat","background-position":"center center","background-size":"auto","background-attachment":"scroll","background-type":"","background-media":"","overlay-type":"","overlay-color":"","overlay-gradient":""}},"ast-content-background-meta":{"desktop":{"background-color":"var(--ast-global-color-5)","background-image":"","background-repeat":"repeat","background-position":"center center","background-size":"auto","background-attachment":"scroll","background-type":"","background-media":"","overlay-type":"","overlay-color":"","overlay-gradient":""},"tablet":{"background-color":"var(--ast-global-color-5)","background-image":"","background-repeat":"repeat","background-position":"center center","background-size":"auto","background-attachment":"scroll","background-type":"","background-media":"","overlay-type":"","overlay-color":"","overlay-gradient":""},"mobile":{"background-color":"var(--ast-global-color-5)","background-image":"","background-repeat":"repeat","background-position":"center center","background-size":"auto","background-attachment":"scroll","background-type":"","background-media":"","overlay-type":"","overlay-color":"","overlay-gradient":""}},"footnotes":""},"categories":[1,380,340,457],"tags":[],"publishpress_future_action":{"enabled":false,"date":"2024-05-12 11:01:31","action":"change-status","newStatus":"draft","terms":[],"taxonomy":"category"},"_links":{"self":[{"href":"https:\/\/idibell.cat\/wp-json\/wp\/v2\/posts\/8540"}],"collection":[{"href":"https:\/\/idibell.cat\/wp-json\/wp\/v2\/posts"}],"about":[{"href":"https:\/\/idibell.cat\/wp-json\/wp\/v2\/types\/post"}],"author":[{"embeddable":true,"href":"https:\/\/idibell.cat\/wp-json\/wp\/v2\/users\/6"}],"replies":[{"embeddable":true,"href":"https:\/\/idibell.cat\/wp-json\/wp\/v2\/comments?post=8540"}],"version-history":[{"count":2,"href":"https:\/\/idibell.cat\/wp-json\/wp\/v2\/posts\/8540\/revisions"}],"predecessor-version":[{"id":19877,"href":"https:\/\/idibell.cat\/wp-json\/wp\/v2\/posts\/8540\/revisions\/19877"}],"wp:featuredmedia":[{"embeddable":true,"href":"https:\/\/idibell.cat\/wp-json\/wp\/v2\/media\/19876"}],"wp:attachment":[{"href":"https:\/\/idibell.cat\/wp-json\/wp\/v2\/media?parent=8540"}],"wp:term":[{"taxonomy":"category","embeddable":true,"href":"https:\/\/idibell.cat\/wp-json\/wp\/v2\/categories?post=8540"},{"taxonomy":"post_tag","embeddable":true,"href":"https:\/\/idibell.cat\/wp-json\/wp\/v2\/tags?post=8540"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}
\nMorat\u00f3 L., Galino J., Ruiz M., Ylagan Calingasan N., Tarrkov A.A., Dumont M., Naud\u00ed A., Mart\u00ednez J.J., Aubourg P, Portero-Ot\u00edn M., Pamplona R., Galea E., Flint Beal M., Ferrer I., Fourcade S. And Pujol A. Pioglitazone halts axonal degeneration in a mouse model of X-linked adrenoleukodystrophy. Brain (2013).<\/p>\n","protected":false},"excerpt":{"rendered":"