Skeletal muscle pathology in X chromosome-linked muscular dystrophy (mdx) mouse

Histological, histochemical, and morphometric analyses were performed chronologically on muscles from mutant mice with X chromosome-linked muscular dystrophy (mdx), and the findings were compared with those in nondystrophic control animals (C57BL/10ScSn). Massive grouped muscle fiber destruction, fo...

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Published in:Acta neuropathologica Vol. 69; no. 1-2; pp. 91 - 95
Main Authors: Tanabe, Y, Esaki, K, Nomura, T
Format: Journal Article
Language:English
Published: Germany 01-01-1986
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Abstract Histological, histochemical, and morphometric analyses were performed chronologically on muscles from mutant mice with X chromosome-linked muscular dystrophy (mdx), and the findings were compared with those in nondystrophic control animals (C57BL/10ScSn). Massive grouped muscle fiber destruction, followed by complete regeneration, occurred abruptly at 20 days of age. There were no preceding changes in body weight, the number and mean diameter of fibers, and fiber type differentiation before the initial episode of muscle fiber necrosis. Muscle fiber necrosis decreased in intensity after 60 days of age. Even after repeated muscle fiber necrosis and regeneration, the most striking finding was that interstitial fibrosis and adipose tissue replacement were minimal, and there was no apparent fiber loss. Since the necrosis was probably well compensated by the active regenerative process, the mdx mice developed no obvious muscle weakness and thus differed from human and other animal muscular dystrophies with the exception of the dystrophic hamster.
AbstractList Histological, histochemical, and morphometric analyses were performed chronologically on muscles from mutant mice with X chromosome-linked muscular dystrophy (mdx), and the findings were compared with those in nondystrophic control animals (C57BL/10ScSn). Massive grouped muscle fiber destruction, followed by complete regeneration, occurred abruptly at 20 days of age. There were no preceding changes in body weight, the number and mean diameter of fibers, and fiber type differentiation before the initial episode of muscle fiber necrosis. Muscle fiber necrosis decreased in intensity after 60 days of age. Even after repeated muscle fiber necrosis and regeneration, the most striking finding was that interstitial fibrosis and adipose tissue replacement were minimal, and there was no apparent fiber loss. Since the necrosis was probably well compensated by the active regenerative process, the mdx mice developed no obvious muscle weakness and thus differed from human and other animal muscular dystrophies with the exception of the dystrophic hamster.
Author Nomura, T
Esaki, K
Tanabe, Y
Author_xml – sequence: 1
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  surname: Tanabe
  fullname: Tanabe, Y
– sequence: 2
  givenname: K
  surname: Esaki
  fullname: Esaki, K
– sequence: 3
  givenname: T
  surname: Nomura
  fullname: Nomura, T
BackLink https://www.ncbi.nlm.nih.gov/pubmed/3962599$$D View this record in MEDLINE/PubMed
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PublicationCentury 1900
PublicationDate 1986-01-01
PublicationDateYYYYMMDD 1986-01-01
PublicationDate_xml – month: 01
  year: 1986
  text: 1986-01-01
  day: 01
PublicationDecade 1980
PublicationPlace Germany
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PublicationTitle Acta neuropathologica
PublicationTitleAlternate Acta Neuropathol
PublicationYear 1986
References I Nonaka (CR13) 1982
FL Mastaglia (CR9) 1970; 11
J Dangain (CR4) 1984; 7
P Wirtz (CR14) 1983; 137
G Karpati (CR7) 1979; 317
AM Michelson (CR10) 1955; 41
F Homburger (CR6) 1962; 6
VS Asmundson (CR2) 1966; 138
I Nonaka (CR11) 1981; 55
I Nonaka (CR12) 1981; 4
CR Ashmore (CR1) 1971; 30
G Bulfield (CR3) 1984; 81
V Dubowitz (CR5) 1973
G Karpati (CR8) 1982; 5
References_xml – volume: 7
  start-page: 700
  year: 1984
  ident: CR4
  publication-title: Muscle Nerve
  doi: 10.1002/mus.880070903
  contributor:
    fullname: J Dangain
– volume: 137
  start-page: 127
  year: 1983
  ident: CR14
  publication-title: J Anat
  contributor:
    fullname: P Wirtz
– volume: 41
  start-page: 1079
  year: 1955
  ident: CR10
  publication-title: Proc Natl Acad Sci USA
  doi: 10.1073/pnas.41.12.1079
  contributor:
    fullname: AM Michelson
– volume: 81
  start-page: 1189
  year: 1984
  ident: CR3
  publication-title: Proc Natl Acad Sci USA
  doi: 10.1073/pnas.81.4.1189
  contributor:
    fullname: G Bulfield
– volume-title: Muscle biopsy. A modern approach
  year: 1973
  ident: CR5
  contributor:
    fullname: V Dubowitz
– volume: 11
  start-page: 425
  year: 1970
  ident: CR9
  publication-title: J Neurol Sci
  doi: 10.1016/0022-510X(70)90002-X
  contributor:
    fullname: FL Mastaglia
– volume: 55
  start-page: 173
  year: 1981
  ident: CR11
  publication-title: Acta Neuropathol (Berl)
  doi: 10.1007/BF00691315
  contributor:
    fullname: I Nonaka
– volume: 138
  start-page: 5
  year: 1966
  ident: CR2
  publication-title: Ann NY Acad Sci
  doi: 10.1111/j.1749-6632.1966.tb41153.x
  contributor:
    fullname: VS Asmundson
– volume: 6
  start-page: 339
  year: 1962
  ident: CR6
  publication-title: Med Exp
  contributor:
    fullname: F Homburger
– start-page: 63
  volume-title: Muscular dystrophy
  year: 1982
  ident: CR13
  contributor:
    fullname: I Nonaka
– volume: 317
  start-page: 89
  year: 1979
  ident: CR7
  publication-title: Ann NY Acad Sci
  doi: 10.1111/j.1749-6632.1979.tb56513.x
  contributor:
    fullname: G Karpati
– volume: 5
  start-page: 369
  year: 1982
  ident: CR8
  publication-title: Muscle Nerve
  doi: 10.1002/mus.880050506
  contributor:
    fullname: G Karpati
– volume: 30
  start-page: 431
  year: 1971
  ident: CR1
  publication-title: Exp Neurol
  doi: 10.1016/0014-4886(71)90144-0
  contributor:
    fullname: CR Ashmore
– volume: 4
  start-page: 326
  year: 1981
  ident: CR12
  publication-title: Muscle Nerve
  doi: 10.1002/mus.880040409
  contributor:
    fullname: I Nonaka
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Snippet Histological, histochemical, and morphometric analyses were performed chronologically on muscles from mutant mice with X chromosome-linked muscular dystrophy...
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StartPage 91
SubjectTerms Animals
Body Weight
Cell Count
Genetic Linkage
Mice
Mice, Inbred C57BL
Muscles - pathology
Muscular Dystrophy, Animal - diagnosis
Muscular Dystrophy, Animal - genetics
Muscular Dystrophy, Animal - pathology
X Chromosome
Title Skeletal muscle pathology in X chromosome-linked muscular dystrophy (mdx) mouse
URI https://www.ncbi.nlm.nih.gov/pubmed/3962599
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