Cookies on this website

We use cookies to ensure that we give you the best experience on our website. If you click 'Accept all cookies' we'll assume that you are happy to receive all cookies and you won't see this message again. If you click 'Reject all non-essential cookies' only necessary cookies providing core functionality such as security, network management, and accessibility will be enabled. Click 'Find out more' for information on how to change your cookie settings.

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 CNS of both DMD children and the mdx mouse model. To date, 31P-magnetic resonance spectroscopy (MRS) has shown in vivo several abnormalities within skeletal muscle of mdx mice and DMD boys. In this study, we determined whether similar abnormalities occur in mdx brain in vivo by using 31P-MRS in addition to metabolite and enzyme analysis to study cerebral metabolism. An increased inorganic phosphate (P(i))/phosphocreatine (PCr) and pH was found in vivo for mdx brain compared with controls, and biochemical analysis showed a reduction in total creatine, an increased extracellular and decreased intracellular volume in mdx brain. No differences were found in any glycolytic or mitochondrial maximal enzyme activities. These changes are discussed with respect to the biochemical changes found in muscle from DMD patients and mdx mice. It is proposed that these biochemical changes may be a factor in the reduced cognitive capacity of mdx mice and some DMD children.

Original publication

DOI

10.1093/brain/119.3.1039

Type

Journal article

Journal

Brain

Publication Date

06/1996

Volume

119 ( Pt 3)

Pages

1039 - 1044

Keywords

Animals, Brain, Cell Size, Disease Models, Animal, Enzymes, Magnetic Resonance Imaging, Mice, Mice, Inbred C57BL, Mice, Inbred mdx, Muscular Dystrophies, Muscular Dystrophy, Animal, Phosphorus Isotopes