Mouse Genome Informatics
involves: FVB/N
phenotype observed in females WTSI Wellcome Trust Sanger Institute
phenotype observed in males EuPh Europhenome
N normal phenotype
• however, mice do not develop muscle weakness or wasting by 6 months of age
• mice exhibit a moderate increase in central nuclei and rare necrotic fibers
• at 18 to 20 days of age, mice exhibit robust myotonic discharges
• myotonia is observed as early as 4 weeks of age when muscle histology is normal
• mice exhibit abnormal hindlimb posture when they initiate movement after long periods of inactivity or when they are suspended by their tails
• 6 of 6 mice display myotonia

Mouse Models of Human Disease
Myotonic Dystrophy 1; DM1 160900 J:64493