Progressive skeletal muscle weakness in transgenic mice expressing CTG expansions is associated with the activation of the ubiquitin–proteasome pathway
A Vignaud, A Ferry, A Huguet, M Baraibar… - Neuromuscular …, 2010 - Elsevier
Myotonic dystrophy type 1 (DM1) is a neuromuscular disease caused by the expansion of a
CTG repeat in the DMPK gene and characterised by progressive skeletal muscle weakness
and wasting. To investigate the effects of the CTG expansion on the physiological function of
the skeletal muscles, we have used a transgenic mouse model carrying the human DM1
region with 550 expanded CTG repeats. Maximal force is reduced in the skeletal muscles of
10-month-old but not in 3-month-old DM1 mice when compared to age-matched non …
CTG repeat in the DMPK gene and characterised by progressive skeletal muscle weakness
and wasting. To investigate the effects of the CTG expansion on the physiological function of
the skeletal muscles, we have used a transgenic mouse model carrying the human DM1
region with 550 expanded CTG repeats. Maximal force is reduced in the skeletal muscles of
10-month-old but not in 3-month-old DM1 mice when compared to age-matched non …