Targeting Myotonic Dystrophy Type 1 with Metformin

Myotonic dystrophy type 1 (DM1) is a multisystemic disorder of genetic origin. Progressive muscular weakness, atrophy and myotonia are its most prominent neuromuscular features, while additional clinical manifestations in multiple organs are also common. Overall, DM1 features resemble accelerated ag...

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Detalhes bibliográficos
Autores: García Puga, Mikel, Sáenz Antoñanzas, Ander, Matheu Fernández, Ander, López de Munain Arregui, Adolfo José
Formato: artículo
Fecha de publicación:2022
País:España
Recursos:Universidad del País Vasco
Repositorio:Addi. Archivo Digital para la Docencia y la Investigación
OAI Identifier:oai:addi.ehu.eus:10810/57789
Acesso em linha:http://hdl.handle.net/10810/57789
Access Level:acceso abierto
Palavra-chave:myotonic dystrophy
metformin
therapeutic strategies
aging
protein-kinase
skeletal-muscle
cancer-risk
trinucleotide repeat
cognitive function
molecular-basis
mouse model
CGT repeat
mortality
expansion
Descrição
Resumo:Myotonic dystrophy type 1 (DM1) is a multisystemic disorder of genetic origin. Progressive muscular weakness, atrophy and myotonia are its most prominent neuromuscular features, while additional clinical manifestations in multiple organs are also common. Overall, DM1 features resemble accelerated aging. There is currently no cure or specific treatment for myotonic dystrophy patients. However, in recent years a great effort has been made to identify potential new therapeutic strategies for DM1 patients. Metformin is a biguanide antidiabetic drug, with potential to delay aging at cellular and organismal levels. In DM1, different studies revealed that metformin rescues multiple phenotypes of the disease. This review provides an overview of recent findings describing metformin as a novel therapy to combat DM1 and their link with aging.