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Duchenne muscular dystrophy (DMD) is an X-linked genetic disorder primarily affecting young boys, often causing mental retardation in addition to the well-known progressive muscular weakness. Normal dystrophin expression is lacking in skeletal muscle and the central nervous system (CNS) of both DMD children and the mdx mouse model. The underlying biochemical lesion causing mental impairment in DMD is unknown. 1H-magnetic resonance spectroscopy (1H-MRS) detects choline-containing compounds, creatine and N-acetyl aspartate (NAA) in vivo. NAA is commonly used as a chemical marker for neurons, and a decline in NAA is thought to correlate with neuronal loss. Control mice were compared to mdx using a combination of in vivo and in vitro 1H-MRS methods to determine whether neural necrosis or developmental abnormalities occur in dystrophic brain. NAA levels were normal in mdx brain compared to controls suggesting minor, if any, neuronal necrosis in dystrophic brain. In contrast, choline compounds and myo-inositol levels were increased, indicative of gliosis or developmental abnormalities in dystrophic brain.

Type

Journal article

Journal

J Neurol Sci

Publication Date

15/09/1996

Volume

141

Pages

13 - 18

Keywords

Animals, Aspartic Acid, Astrocytes, Brain, Brain Chemistry, Choline, Creatine, Dystrophin, Female, Gliosis, Magnetic Resonance Spectroscopy, Male, Mice, Mice, Inbred C57BL, Mice, Inbred mdx, Necrosis, Neurons