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Neuromuscul Disord ; 36: 16-22, 2024 Mar.
Artículo en Inglés | MEDLINE | ID: mdl-38306718

RESUMEN

The European Joint Programme on Rare Diseases (EJPRD) funded the workshop "LAMA2-Muscular Dystrophy: Paving the road to therapy", bringing together 40 health-care professionals, researchers, patient-advocacy groups, Early-Career Scientists and other stakeholders from 14 countries. Progress in natural history, pathophysiology, trial readiness, and treatment strategies was discussed together with efforts to increase patient-awareness and strengthen collaborations. Key outcomes were (a) ongoing natural history studies in 7 countries already covered more than 350 patients. The next steps are to include additional countries, harmonise data collection and define a minimal dataset; (b) therapy development was largely complementary. Approaches included LAMA2-replacement and correction, LAMA1-reactivation, mRNA modulation, linker-protein expression, targeting downstream processes and identifying modifiers, using viral vectors, muscle stem cells, iPSC and mouse models and patient lines; (c) LAMA2-Europe will inform patients (-representatives) worldwide on standards of care and scientific progress, and enable sharing experiences. Follow-up monthly online meetings and research repositories have been established to create sustainable collaborations.


Asunto(s)
Distrofias Musculares , Enfermedades Raras , Animales , Humanos , Ratones , Europa (Continente) , Laminina/genética , Laminina/metabolismo , Distrofias Musculares/genética , Distrofias Musculares/terapia , Enfermedades Raras/genética , Enfermedades Raras/terapia , España
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