Properties of slow- and fast-twitch muscle fibres in a mouse model of amyotrophic lateral sclerosis

Atkin, Julie D., Scott, Rachel L., West, Jan, Lopes, Elizabeth, Quah, Alvin K. J. and Cheema, Surindar S. 2005, Properties of slow- and fast-twitch muscle fibres in a mouse model of amyotrophic lateral sclerosis, Neuromuscular disorders, vol. 15, no. 5, pp. 377-388, doi: 10.1016/j.nmd.2005.02.005.

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Title Properties of slow- and fast-twitch muscle fibres in a mouse model of amyotrophic lateral sclerosis
Author(s) Atkin, Julie D.
Scott, Rachel L.
West, JanORCID iD for West, Jan
Lopes, Elizabeth
Quah, Alvin K. J.
Cheema, Surindar S.
Journal name Neuromuscular disorders
Volume number 15
Issue number 5
Start page 377
End page 388
Publisher Elsevier Ltd
Place of publication Oxford, England
Publication date 2005-05
ISSN 0960-8966
Keyword(s) ALS
SOD1 G93A mice
muscle atrophy
skeletal muscle
Summary This investigation was undertaken to determine if there are altered histological, pathological and contractile properties in presymptomatic or endstage diseased muscle fibres from representative slow-twitch and fast-twitch muscles of SOD1 G93A mice in comparison to wildtype mice. In presymptomatic SOD1 G93A mice, there was no detectable peripheral dysfunction, providing evidence that muscle pathology is secondary to motor neuronal dysfunction. At disease endstage however, single muscle fibre contractile analysis demonstrated that fast-twitch muscle fibres and neuromuscular junctions are preferentially affected by amyotrophic lateral sclerosis-induced denervation, being unable to produce the same levels of force when activated by calcium as muscle fibres from their age-matched controls. The levels of transgenic SOD1 expression, aggregation state and activity were also examined in these muscles but there no was no preference for muscle fibre type. Hence, there is no simple correlation between SOD1 protein expression/activity, and muscle fibre type vulnerability in SOD1 G93A mice.
Notes Available online 29 March 2005.
Language eng
DOI 10.1016/j.nmd.2005.02.005
Field of Research 060699 Physiology not elsewhere classified
HERDC Research category C1 Refereed article in a scholarly journal
Copyright notice ©2005, Elsevier B.V.
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