Abstract
The role of dystrophin disorders in the CNS function of boys with Duchenne muscular dystrophy (DMD) and the dystrophin-deficient mdx mouse, an animal model of DMD, is reviewed at the University of New South Wales, University of Sydney, Australia.
Highlights
Two infants born to mothers with pregnancy-related polymyositis (PM) had unusually elevated serum creatine kinase (CK) levels, in a report from the University of Milan, Italy
Brain histology shows abnormalities in dendrites and loss of neurones, and biochemical changes are indicative of CNS pathology, including an increase in levels of choline-containing compounds
Studies in the mdx mouse have shown a disruption of GABA receptors, that may impact on the action of certain drugs, sleep disorders, and motor control in Duchenne muscular dystrophy (DMD)
Summary
Two infants born to mothers with pregnancy-related polymyositis (PM) had unusually elevated serum creatine kinase (CK) levels, in a report from the University of Milan, Italy. Duchenne muscular dystrophy (DMD) and the dystrophin-deficient mdx mouse, an animal model of DMD, is reviewed at the University of New South Wales, University of Sydney, Australia. Brain histology shows abnormalities in dendrites and loss of neurones, and biochemical changes are indicative of CNS pathology, including an increase in levels of choline-containing compounds.
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