Immunostaining of Dystrophin and Utrophin in Skeletal Muscle of Dystrophinopathies.

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概要

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• Immunostaining of biopsied skeletal muscle of 4 Duchenne (DMD), 12 Becker muscular dystrophy (BMD) and 3 DMD carriers' was performed using monoclonal antibodies against dystrophin and utrophin. In DMD, dystrophin-negative staining was observed except for revertant fibers which showed different stain patterns for each antibody. In 7 BMDs, there was faint/patchy stain in cases of deletion between exons 45-52, while in one case there was deletion between exons 12-17 and no stain was noted relevant to the deletion site. Moreover, in 2 cases of undetectable deletion, antibodies which recognize a terminal portion of the C-terminal domain revealed the absent stain. In DMD, the utrophin-positive fibers corresponded to dystrophin-negative fibers. In BMD, this relationship did not necessarily occur in each fiber. In DMD carriers, a cluster of dystrophin-negative fibers which was positive for utrophin were prominent. In dystrophinopathy, the immunostaining of dystrophin and utrophin is useful, in combination with dystrophin gene analysis to make a definite diagnosis.(Internal Medicine 33: 277-283, 1994)

• 社団法人　日本内科学会の論文

著者

• Sugita Hideo

  National Center Neurology And Psychiatry

• Arahata Kiichi

  National Institute Of Neuroscience Ncnp

• NAKAO Naoki

  Neurology Section, Fourth Department of Medicine, Aichi Medical University

• SAHASHI Ko

  Neurology Section, Fourth Department of Medicine, Aichi Medical University

• IBI Tohru

  Neurology Section, Fourth Department of Medicine, Aichi Medical University

• SUOH Hiroshi

  Neurology Section, Fourth Department of Medicine, Aichi Medical University

• TASHIRO Michinari

  Neurology Section, Fourth Department of Medicine, Aichi Medical University

• MARUI Kiminori

  Neurology Section, Fourth Department of Medicine, Aichi Medical University

• ARAHATA Kiichi

  National Center of Neurology and Psychiatry, National Institute of Neuroscience

• SUGITA Hideo

  National Center for Nervous, Mental and Muscular Disorders

関連論文

• Progress in muscular dystrophy research with special emphasis on gene therapy
• A carrier of Duchenne muscular dystrophy with dilated cardiomyopathy but no skeletal muscle symptom
• Nationwide Survey of Neurologic Manifestations of Acquired Immunodeficiency Syndrome in Japan
• Laminin in Animal Models for Muscular Dystrophy Defect of Laminin M in Skeletal and Cardiac Muscles and Peripheral Nerve of the Homozygous Dystrophic dy/dy Mice
• Molecular diagnosis of muscular dystrophies
• Porcine Skeletal Muscle Dystrophin
• Immunostaining of Dystrophin and Utrophin in Skeletal Muscle of Dystrophinopathies.
• Anti-filamin and -vinculin antibodies in sera from patients with myasthenia gravis and polymyositis.
• Progressive External Ophthalmoplegia and Myositis.
• Negative immunostaining of Duchenne muscular dystrophy(DMD) and mdx muscle surface membrane with antibody against synthetic peptide fragment predicted from DMD cDNA.
• A Dystrophin Homologue on the Surface Membrane of Embryonic and Denervated mdx Mouse Muscle Fibers.
• Human skeletal muscle contains two major aminopeptidases: An anion-activated aminopeptidase B and an aminopeptidase M-like enzyme.

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