Hereditary muscular dystrophy in MDX mice as a homologous model for introduction of cell technologies in the treatment of progressive muscular dystrophies in humans.
Life-time monitoring of the main clinical and laboratory manifestations of hereditary muscular dystrophy in mdx mice confirmed the presence of mutation in exon 23 of dystrophin gene and the absence of this protein in skeletal muscles of mutant animals. Muscular dystrophy in mice was similar to human progressive muscle disorder, which allows the use of this model for the development of cell technologies for the treatment of hereditary muscular diseases in humans.
Stenina
M A
MA
Russian State Medical University.
Savchuk
V I
VI
Sitnikov
V F
VF
Krivov
L I
LI
Kuznetsov
A B
AB
Voevodin
D A
DA
Yarygin
V N
VN
Sukhikh
G T
GT
D000818:Animals; D016189:Dystrophin / Q000235:genetics / Q000378:metabolism; D005091:Exons; D006801:Humans; D008297:Male; D051379:Mice; D018101:Mice, Inbred mdx; D009043:Motor Activity; D018482:Muscle, Skeletal / Q000473:pathology / Q000503:physiopathology; D009137:Muscular Dystrophy, Animal / Q000235:genetics* / Q000473:pathology / Q000503:physiopathology / Q000628:therapy*; D017354:Point Mutation
D016189:Dystrophin