Investigadors de l’Institut d’Investigaci\u00f3 Biom\u00e8dica de Bellvitge (IDIBELL) a la Universitat de Barcelona (UB) han coordinat una investigaci\u00f3 sobre com el nitril IDPN provoca s\u00edndromes neurol\u00f2giques semblants als de l’esclerosi lateral amiotr\u00f2fica (ELA), una greu malaltia degenerativa neuromuscular. Aquest estudi, dirigit per Jordi Llorens, ha estat publicat recentment a la revista Neuropathology and Applied Neurobiology.<\/p>\n
Els nitrils, compostos qu\u00edmics que contenen el grup ci\u00e0 (-CN), s\u00f3n molt abundants en la natura i tenen diverses aplicacions en la ind\u00fastria. En la natura es troben en forma de gluc\u00f2sids cianog\u00e8nics, per exemple en les ametlles amargues, i en forma d\u2019aminonitrils, en algunes lleguminoses. En la ind\u00fastria s’utilitzen com a dissolvents i productes intermediaris en la s\u00edntesi de pl\u00e0stics, fibres artificials, resines i productes farmac\u00e8utics, entre d’altres. El consum hum\u00e0 o animal de determinats nitrils provoca s\u00edmptomes similars a la intoxicaci\u00f3 per cianur. Aquest fet suggereix que en la metabolitzaci\u00f3 d’aquests nitrils es produeix l’alliberament d’aquest compost, sent el responsable de la intoxicaci\u00f3 aguda. Alguns nitrils, alliberen menys cianur o ho fan de forma m\u00e9s lenta i aquests s\u00f3n els que provoquen neurotoxicitat i s\u00edndromes neurol\u00f2giques.<\/p>\n
En una gran varietat de malalties del sistema nervi\u00f3s apareix una acumulaci\u00f3 anormal de neurofilaments, fibres que confereixen rigidesa a les neurones. Concretament, en l’esclerosi lateral amiotr\u00f2fica (ELA) s’observen protuber\u00e0ncies formades per una gran quantitat de neurofilaments en els axons de les neurones motores.<\/p>\n
En l’estudi, els investigadors han observat acumulacions de neurofilaments sorprenentment similars als que apareixen a l’ELA, en rates de laboratori exposades a l\u2019IDPN (3,3′-iminodipropionitril). A causa d’aquesta similitud, s’ha estudiat l’efecte de l\u2019IDPN per comprendre la biologia dels neurofilaments en la malaltia de l’ELA. Tant la malaltia com la intoxicaci\u00f3 per IDPN provoquen axonopatia, una lesi\u00f3 dels axons. La novetat de l’estudi \u00e9s l’observaci\u00f3 que l\u2019axonopatia provoca una p\u00e8rdua marcada de neurofilaments en els terminals de les neurones motores. Aquesta observaci\u00f3 \u00e9s rellevant ja que la refracci\u00f3 dels terminals \u00e9s el factor cr\u00edtic en la degeneraci\u00f3 de la neurona motora.<\/p>\n
El coordinador de la recerca explica que “la manca de neurofilaments a la zona d\u2019uni\u00f3 neuromuscular pot tenir un impacte en la funci\u00f3 o l’estabilitat d’aquesta uni\u00f3, contribuint a la seva retracci\u00f3 i la posterior regeneraci\u00f3 de la neurona”, conclou Llorens.<\/p>\n
Potencials aplicacions cl\u00edniques<\/strong><\/p>\n A causa de la similitud entre les acumulacions proximals de neurofilaments entre el model animal d\u2019IDPN i l’ELA, els investigadors preveuen que les unions neuromusculars dels pacients d’esclerosi lateral amiotr\u00f2fica mostraran una falta de neurofilaments similar a l’observada en el model animal. Aquesta p\u00e8rdua de neurofilaments terminals podria ser un factor patog\u00e8nic en la malaltia.<\/p>\n Cada vegada \u00e9s m\u00e9s evident que l’ELA \u00e9s una malaltia multifactorial, en la qual influeixen factors gen\u00e8tics i ambientals que causen una axonopatia proximal. Aquests factors podrien alterar la uni\u00f3 neuromuscular a trav\u00e9s del mecanisme de buidatge de neurofilaments observat en la intoxicaci\u00f3 per IDPN. A m\u00e9s, les dades obtingudes indiquen que l\u2019IDPN sembla una eina \u00fatil per a ser inclosa en models animals multifactorials de la malaltia.<\/p>\n L’estudi s’ha presentat divendres passat a Barcelona durant el simposi “Mechanisms of neurotoxicity and Implications for Neurological Disorders”, activitat sat\u00e8l\u00b7lit del congr\u00e9s de la Federaci\u00f3 Europea de Neuroci\u00e8ncia (FENS).<\/p>\n Refer\u00e8ncia de l’article: <\/strong>Soler-Mart\u00edn C, Vilardosa U, Salda\u00f1a-Ru\u00edz S, Garcia N, Llorens J. Loss of neurofilaments in the neuromuscular junction in a rat model of proximal axonopathy. Neuropathology and Applied Neurobiology 2012 febrer; 38 (1) :61-71.<\/p>\n","protected":false},"excerpt":{"rendered":" Investigadors de l’Institut d’Investigaci\u00f3 Biom\u00e8dica de Bellvitge (IDIBELL) a la Universitat de Barcelona (UB) han coordinat una investigaci\u00f3 sobre com el nitril IDPN provoca s\u00edndromes neurol\u00f2giques semblants als de l’esclerosi lateral amiotr\u00f2fica (ELA), una greu malaltia degenerativa neuromuscular. Aquest estudi, dirigit per Jordi Llorens, ha estat publicat recentment a la revista Neuropathology and Applied Neurobiology. 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