The neurobiology of Duchenne muscular dystrophy: learning lessons from muscle?

Several forms of inherited muscular dystrophy are associated with brain abnormalities and cognitive impairment. One of the most common and severe of these diseases is Duchenne muscular dystrophy (DMD). Dystrophin, the product of the DMD gene, is found in neurones, where it is associated with the pos...

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Bibliographic Details
Published in:Trends in neurosciences (Regular ed.) 2000-03, Vol.23 (3), p.92-99
Main Authors: Blake, Derek J., Kröger, Stephan
Format: Article
Language:English
Subjects:
Animals
Biological and medical sciences
Biology
Brain - metabolism
Brain - physiopathology
Cognition Disorders - etiology
Cognition Disorders - metabolism
Diseases of striated muscles. Neuromuscular diseases
Dystroglycan
Dystrophin
Dystrophin - deficiency
Dystrophin - genetics
Dystrophin - metabolism
Genetics
Humans
Medical sciences
Mice
Multigene Family
Muscle, Skeletal - metabolism
Muscle, Skeletal - pathology
Muscular dystrophy
Muscular Dystrophy, Duchenne - complications
Muscular Dystrophy, Duchenne - genetics
Muscular Dystrophy, Duchenne - metabolism
Muscular Dystrophy, Duchenne - physiopathology
Muscular system
Neurology
Neuronal nitric oxide
Neurons - metabolism
Nitric Oxide - biosynthesis
Retina
Retina - physiopathology
Synapse
Synaptic Membranes - metabolism
Synthase
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Summary:Several forms of inherited muscular dystrophy are associated with brain abnormalities and cognitive impairment. One of the most common and severe of these diseases is Duchenne muscular dystrophy (DMD). Dystrophin, the product of the DMD gene, is found in neurones, where it is associated with the postsynaptic membrane. Cognitive impairment in individuals with DMD is thought to be due to an abnormality in the neuronal membrane that is caused by lack of dystrophin. Recent experimental evidence has provided valuable clues in our understanding of the complex molecular neurobiology of muscular dystrophy.
ISSN:0166-2236
1878-108X
DOI:10.1016/S0166-2236(99)01510-6