Mouse Genome Informatics
hm
    Lama2tm1Stk/Lama2tm1Stk
involves: 129/Sv * 129P2/OlaHsd * BALB/c * ICR
Key:
phenotype observed in females WTSI Wellcome Trust Sanger Institute
phenotype observed in males EuPh Europhenome
N normal phenotype
       
mortality/aging
• mice die of undetermined causes by 5 weeks of age

muscle
• by P21 and P28, proliferation of endomysial connective tissue is observed
• the number of apoptotic nuclei in muscle fibers is increased compared to in wild-type mice
• by P21 and P28, muscle fibers of different calibers are apparent
• by P24 mice exhibit dystrophic symptoms including a waddling gait and twitching
• by P24 mice exhibit twitching
• by P9 mice exhibit scattered degeneration of muscle fibers and at P11 numerous degenerating fibers with extensive infiltrate are observed
• degeneration is accompanied by regeneration at P13

behavior/neurological
• by P24 mice exhibit a waddling gait

growth/size
• beginning at P14 and continuing for the rest of their lives
• apparent after postnatal day 14

cellular
• the basal lamina is nearly completely absent around muscle fibers and Schwann cells of peripheral nerves

Mouse Models of Human Disease
OMIM IDRef(s)
Muscular Dystrophy, Congenital Merosin-Deficient, 1A; MDC1A 607855 J:43145 , J:167230