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High resolution magnetic resonance imaging of the brain in the dy/dy mouse with merosin-deficient congenital muscular dystrophy

Dubowitz, David J. and Tyszka, J. Michael and Sewry, Caroline A. and Moats, Rex A. and Scadeng, Miriam and Dubowitz, Victor (2000) High resolution magnetic resonance imaging of the brain in the dy/dy mouse with merosin-deficient congenital muscular dystrophy. Neuromuscular Disorders, 10 (4-5). pp. 292-298. ISSN 0960-8966. doi:10.1016/S0960-8966(00)00104-8.

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Magnetic resonance imaging (MRI) abnormalities in the cerebral white matter are a consistent feature of merosin-deficient human congenital muscular dystrophy, a disease caused by a primary defect in the expression of the laminin alpha2 chain of merosin. To investigate the relationship between imaging changes and merosin deficiency we undertook a MRI study in the dy/dy mouse, an animal model for this form of human congenital muscular dystrophy. High resolution in vivo imaging was performed on anaesthetized animals (two homozygous dy/dy mutants and two heterozygous dy/DY controls, aged 2.5 months) in a dedicated 11.7T magnetic resonance imaging scanner. T(1) and T(2) weighted images were normal in all mice and white matter changes were not seen at a stage of maturity when MRI changes are already very striking in human patients. Cerebral MRI abnormalities do not appear to be a feature of dy/dy mice, despite the virtual absence of merosin expression in the dy/dy mouse brain. Possible causes for this absence of MRI changes, and implications for the pathogenesis of the MRI changes in humans are reviewed.

Item Type:Article
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URLURL TypeDescription DOIArticle
Tyszka, J. Michael0000-0001-9342-9014
Additional Information:© 2000 Elsevier Science B.V. Received 21 September 1999; received in revised form 13 January 2000; accepted 31 January 2000. The authors wish to acknowledge grant support from the Pasadena Neurosciences Fellowship of Huntington Medical Research Institutes (D.J.D.), and the Beckman Institute, California Institute of Technology. The Human Brain Project, with contributions from the National Institute on Drug Abuse and the National Institute of Mental Health. The authors are grateful to Dr Janet Baer DVM for veterinary assistance and advice throughout this study. Ethical approval for this research was obtained from The Institutional Animal Care and Use Committee of California Institute of Technology.
Funding AgencyGrant Number
Huntington Medical Research Institute (HMRI)UNSPECIFIED
Caltech Beckman InstituteUNSPECIFIED
National Institute on Drug Abuse (NIDA)UNSPECIFIED
National Institute of Mental Health (NIMH)UNSPECIFIED
Subject Keywords:Magnetic resonance imaging; Congenital muscular dystrophy; dy/dy mouse; Laminin α2; merosin
Issue or Number:4-5
Record Number:CaltechAUTHORS:20150908-164247490
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Official Citation:David J Dubowitz, J.Michael Tyszka, Caroline A Sewry, Rex A Moats, Miriam Scadeng, Victor Dubowitz, High resolution magnetic resonance imaging of the brain in the dy/dy mouse with merosin-deficient congenital muscular dystrophy, Neuromuscular Disorders, Volume 10, Issues 4–5, 1 June 2000, Pages 292-298, ISSN 0960-8966, (
Usage Policy:No commercial reproduction, distribution, display or performance rights in this work are provided.
ID Code:60108
Deposited By: Julian Tyszka
Deposited On:10 Sep 2015 20:44
Last Modified:10 Nov 2021 22:29

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