The influence of muscle type and dystrophin deficiency on murine expression profiles |
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Authors: | Judith N Haslett Peter B Kang Mei Han Alvin T Kho Despina Sanoudou Jay M Volinski Alan H Beggs Isaac S Kohane Louis M Kunkel |
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Institution: | (1) Division of Genetics and Genomics Program, Children’s Hospital Boston and Harvard Medical School, Boston, Massachusetts 02115, USA;(2) Department of Neurology, Children’s Hospital Boston and Harvard Medical School, Boston, Massachusetts 02115, USA;(3) Informatics Program, Children’s Hospital Boston and Harvard Medical School, Boston, Massachusetts 02115, USA;(4) Division of Endocrinology, Children’s Hospital Boston and Harvard Medical School, Boston, Massachusetts 02115, USA;(5) Howard Hughes Medical Institute, 4000 Jones Bridge Road, Chevy Chase, Maryland 20815, USA |
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Abstract: | The phenotypic differences among Duchenne muscular dystrophy patients, mdx mice, and mdx5cv mice suggest that despite the common etiology of dystrophin deficiency, secondary mechanisms have a substantial influence
on phenotypic severity. The differential response of various skeletal muscles to dystrophin deficiency supports this hypothesis.
To explore these differences, gene expression profiles were generated from duplicate RNA targets extracted from six different
skeletal muscles (diaphragm, soleus, gastrocnemius, quadriceps, tibialis anterior, and extensor digitorum longus) from wild-type,
mdx, and mdx5cv mice, resulting in 36 data sets for 18 muscle samples. The data sets were compared in three different ways: (1) among wild-type
samples only, (2) among all 36 data sets, and (3) between strains for each muscle type. The molecular profiles of soleus and
diaphragm separate significantly from the other four muscle types and from each other. Fiber-type proportions can explain
some of these differences. These variations in wild-type gene expression profiles may also reflect biomechanical differences
known to exist among skeletal muscles. Further exploration of the genes that most distinguish these muscles may help explain
the origins of the biomechanical differences and the reasons why some muscles are more resistant than others to dystrophin
deficiency.
Electronic Supplementary Material Electronic Supplementary material is available for this article at
and accessible for authorised users.
Judith N. Haslett, Peter B. Kang These authors contributed equally to this work. |
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