PMID- 2852214
OWN - NLM
STAT- MEDLINE
DCOM- 19890329
LR  - 20190724
IS  - 0022-510X (Print)
IS  - 0022-510X (Linking)
VI  - 88
IP  - 1-3
DP  - 1988 Dec
TI  - Perifascicular atrophic fibers in childhood dermatomyositis with particular 
      reference to mitochondrial changes.
PG  - 133-43
AB  - Ten of 17 muscle biopsy specimens from 15 patients with childhood dermatomyositis 
      (DM) showed distinct perifascicular atrophy. The atrophic fibers showed the 
      following characteristics: (1) decreased cytochrome c oxidase (CCO) activity, (2) 
      rare positive reaction with acridine orange (AO) staining, (3) type 2C reaction 
      in 7.9% (0.4-17.5%) of the fibers, (4) an increased number of activated satellite 
      cells, (5) mitochondria which were increased in number but decreased in size, (6) 
      a significantly decreased CCO activity in isolated mitochondria (51.6 +/- 30.3 
      nmol/min per mg mitochondrial protein) as compared with that in the controls 
      (103.6 +/- 41.5). The major pathogenetic mechanism in muscles in childhood DM is 
      thought to be ischemia due to involvement of the microvasculature. The presence 
      of type 2C fibers and increased numbers of activated satellite cells reflect a 
      focal repair process taking place concomitantly in the damaged myofibers. 
      Mitochondrial enzyme defect, especially CCO deficiency is present not only in 
      genetic disorders with mitochondrial involvement but in other neuromuscular 
      disorders including inflammatory myopathies.
FAU - Woo, M
AU  - Woo M
AD  - Division of Ultrastructural Research, National Institute of Neuroscience, NCNP, 
      Tokyo, Japan.
FAU - Chung, S J
AU  - Chung SJ
FAU - Nonaka, I
AU  - Nonaka I
LA  - eng
PT  - Journal Article
PL  - Netherlands
TA  - J Neurol Sci
JT  - Journal of the neurological sciences
JID - 0375403
RN  - EC 1.- (Succinate Cytochrome c Oxidoreductase)
RN  - EC 1.6.99.3 (NADH Dehydrogenase)
RN  - EC 1.9.3.1 (Electron Transport Complex IV)
SB  - IM
MH  - Age Factors
MH  - Atrophy
MH  - Biopsy
MH  - Child
MH  - Child, Preschool
MH  - Dermatomyositis/enzymology/*pathology
MH  - Electron Transport Complex IV/metabolism
MH  - Female
MH  - Humans
MH  - Male
MH  - Mitochondria, Muscle/enzymology/*ultrastructure
MH  - NADH Dehydrogenase/metabolism
MH  - Succinate Cytochrome c Oxidoreductase/metabolism
EDAT- 1988/12/01 00:00
MHDA- 1988/12/01 00:01
CRDT- 1988/12/01 00:00
PHST- 1988/12/01 00:00 [pubmed]
PHST- 1988/12/01 00:01 [medline]
PHST- 1988/12/01 00:00 [entrez]
AID - 0022-510X(88)90211-0 [pii]
AID - 10.1016/0022-510x(88)90211-0 [doi]
PST - ppublish
SO  - J Neurol Sci. 1988 Dec;88(1-3):133-43. doi: 10.1016/0022-510x(88)90211-0.