Intrinsic laryngeal muscles are spared from myonecrosis in the mdx mouse model of Duchenne muscular dystrophy

Intrinsic laryngeal muscles share many anatomical and physiological properties with extraocular muscles, which are unaffected in both Duchenne muscular dystrophy and mdx mice. We hypothesized that intrinsic laryngeal muscles are spared from myonecrosis in mdx mice and may serve as an additional tool...

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Veröffentlicht in:Muscle & nerve 2007-03, Vol.35 (3), p.349-353
Hauptverfasser: Marques, Maria Julia, Ferretti, Renato, Vomero, Viviane Urbini, Minatel, Elaine, Neto, Humberto Santo
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container_start_page 349
container_title Muscle & nerve
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creator Marques, Maria Julia
Ferretti, Renato
Vomero, Viviane Urbini
Minatel, Elaine
Neto, Humberto Santo
description Intrinsic laryngeal muscles share many anatomical and physiological properties with extraocular muscles, which are unaffected in both Duchenne muscular dystrophy and mdx mice. We hypothesized that intrinsic laryngeal muscles are spared from myonecrosis in mdx mice and may serve as an additional tool to understand the mechanisms of muscle sparing in dystrophinopathy. Intrinsic laryngeal muscles and tibialis anterior (TA) muscle of adult and aged mdx and control C57Bl/10 mice were investigated. The percentage of central nucleated fibers, as a sign of muscle fibers that had undergone injury and regeneration, and myofiber labeling with Evans blue dye, as a marker of myofiber damage, were studied. Except for the cricothyroid muscle, none of the intrinsic laryngeal muscles from adult and old mdx mice showed signs of myofiber damage or Evans blue dye labeling, and all appeared to be normal. Central nucleation was readily visible in the TA of the same mdx mice. A significant increase in the percentage of central nucleated fibers was observed in adult cricothyroid muscle compared to the other intrinsic laryngeal muscles, which worsened with age. Thus, we have shown that the intrinsic laryngeal muscles are spared from the lack of dystrophin and may serve as a useful model to study the mechanisms of muscle sparing in dystrophinopathy. Muscle Nerve, 2006
doi_str_mv 10.1002/mus.20697
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We hypothesized that intrinsic laryngeal muscles are spared from myonecrosis in mdx mice and may serve as an additional tool to understand the mechanisms of muscle sparing in dystrophinopathy. Intrinsic laryngeal muscles and tibialis anterior (TA) muscle of adult and aged mdx and control C57Bl/10 mice were investigated. The percentage of central nucleated fibers, as a sign of muscle fibers that had undergone injury and regeneration, and myofiber labeling with Evans blue dye, as a marker of myofiber damage, were studied. Except for the cricothyroid muscle, none of the intrinsic laryngeal muscles from adult and old mdx mice showed signs of myofiber damage or Evans blue dye labeling, and all appeared to be normal. Central nucleation was readily visible in the TA of the same mdx mice. A significant increase in the percentage of central nucleated fibers was observed in adult cricothyroid muscle compared to the other intrinsic laryngeal muscles, which worsened with age. 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Psychology ; intrinsic laryngeal muscles ; Laryngeal Muscles - pathology ; Laryngeal Muscles - physiopathology ; Male ; mdx mouse ; Medical sciences ; Mice ; Mice, Inbred C57BL ; Mice, Inbred mdx ; Muscle Fibers, Skeletal - metabolism ; Muscle Fibers, Skeletal - pathology ; muscle regeneration ; Muscular Dystrophy, Duchenne - pathology ; Muscular Dystrophy, Duchenne - physiopathology ; Necrosis - genetics ; Necrosis - pathology ; Necrosis - physiopathology ; Neurology ; Regeneration - physiology ; Striated muscle. 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Psychology</subject><subject>intrinsic laryngeal muscles</subject><subject>Laryngeal Muscles - pathology</subject><subject>Laryngeal Muscles - physiopathology</subject><subject>Male</subject><subject>mdx mouse</subject><subject>Medical sciences</subject><subject>Mice</subject><subject>Mice, Inbred C57BL</subject><subject>Mice, Inbred mdx</subject><subject>Muscle Fibers, Skeletal - metabolism</subject><subject>Muscle Fibers, Skeletal - pathology</subject><subject>muscle regeneration</subject><subject>Muscular Dystrophy, Duchenne - pathology</subject><subject>Muscular Dystrophy, Duchenne - physiopathology</subject><subject>Necrosis - genetics</subject><subject>Necrosis - pathology</subject><subject>Necrosis - physiopathology</subject><subject>Neurology</subject><subject>Regeneration - physiology</subject><subject>Striated muscle. 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We hypothesized that intrinsic laryngeal muscles are spared from myonecrosis in mdx mice and may serve as an additional tool to understand the mechanisms of muscle sparing in dystrophinopathy. Intrinsic laryngeal muscles and tibialis anterior (TA) muscle of adult and aged mdx and control C57Bl/10 mice were investigated. The percentage of central nucleated fibers, as a sign of muscle fibers that had undergone injury and regeneration, and myofiber labeling with Evans blue dye, as a marker of myofiber damage, were studied. Except for the cricothyroid muscle, none of the intrinsic laryngeal muscles from adult and old mdx mice showed signs of myofiber damage or Evans blue dye labeling, and all appeared to be normal. Central nucleation was readily visible in the TA of the same mdx mice. A significant increase in the percentage of central nucleated fibers was observed in adult cricothyroid muscle compared to the other intrinsic laryngeal muscles, which worsened with age. Thus, we have shown that the intrinsic laryngeal muscles are spared from the lack of dystrophin and may serve as a useful model to study the mechanisms of muscle sparing in dystrophinopathy. Muscle Nerve, 2006</abstract><cop>Hoboken</cop><pub>Wiley Subscription Services, Inc., A Wiley Company</pub><pmid>17143878</pmid><doi>10.1002/mus.20697</doi><tpages>5</tpages></addata></record>
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subjects aging
Aging - pathology
Animals
Biological and medical sciences
Cell Nucleus - pathology
Disease Models, Animal
Disease Progression
Diseases of striated muscles. Neuromuscular diseases
dystrophin
Dystrophin - deficiency
Evans Blue
Fundamental and applied biological sciences. Psychology
intrinsic laryngeal muscles
Laryngeal Muscles - pathology
Laryngeal Muscles - physiopathology
Male
mdx mouse
Medical sciences
Mice
Mice, Inbred C57BL
Mice, Inbred mdx
Muscle Fibers, Skeletal - metabolism
Muscle Fibers, Skeletal - pathology
muscle regeneration
Muscular Dystrophy, Duchenne - pathology
Muscular Dystrophy, Duchenne - physiopathology
Necrosis - genetics
Necrosis - pathology
Necrosis - physiopathology
Neurology
Regeneration - physiology
Striated muscle. Tendons
Vertebrates: osteoarticular system, musculoskeletal system
title Intrinsic laryngeal muscles are spared from myonecrosis in the mdx mouse model of Duchenne muscular dystrophy
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