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

journal contribution
posted on 2005-05-01, 00:00 authored by J Atkin, R Scott, Jan WestJan West, E Lopes, A Quah, S Cheema
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.

History

Journal

Neuromuscular disorders

Volume

15

Issue

5

Pagination

377 - 388

Publisher

Elsevier Ltd

Location

Oxford, England

ISSN

0960-8966

eISSN

1873-2364

Language

eng

Notes

Available online 29 March 2005.

Publication classification

C1 Refereed article in a scholarly journal

Copyright notice

2005, Elsevier B.V.