Effects of metformin on congenital muscular dystrophy type 1A disease progression in mice: a gender impact study

Cibely Fontes Oliveira, Bernardo M. Soares Oliveira, Zandra Körner, Vahid Harandi, Madeleine Durbeej-Hjalt

Research output: Contribution to journalArticlepeer-review

7 Citations (SciVal)

Abstract

Congenital muscular dystrophy with laminin α2 chain-deficiency (LAMA2-CMD) is a severe muscle disorder with complex underlying pathogenesis. We have previously employed profiling techniques to elucidate molecular patterns and demonstrated significant metabolic impairment in skeletal muscle from LAMA2-CMD patients and mouse models. Thus, we hypothesize that skeletal muscle metabolism may be a promising pharmacological target to improve muscle function in LAMA2-CMD. Here, we have investigated whether the multifunctional medication metformin could be used to reduce disease in the dy2J/dy2J mouse model of LAMA2-CMD. First, we show gender disparity for several pathological hallmarks of LAMA2-CMD. Second, we demonstrate that metformin treatment significantly increases weight gain and energy efficiency, enhances muscle function and improves skeletal muscle histology in female dy2J/dy2J mice (and to a lesser extent in dy2J/dy2J males). Thus, our current data suggest that metformin may be a potential future supportive treatment that improves many of the pathological characteristics of LAMA2-CMD.
Original languageEnglish
Article number16302
Number of pages15
JournalScientific Reports
DOIs
Publication statusPublished - 2018 Nov 2

Subject classification (UKÄ)

  • Other Clinical Medicine

Keywords

  • MDC1A; Gender; Metformin

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