Syncoilin modulates peripherin filament networks and is necessary for large-calibre motor neurons.

Published

Journal Article

Syncoilin is an atypical type III intermediate filament (IF) protein, which is expressed in muscle and is associated with the dystrophin-associated protein complex. Here, we show that syncoilin is expressed in both the central and peripheral nervous systems. Isoform Sync1 is dominant in the brain, but isoform Sync2 is dominant in the spinal cord and sciatic nerve. Peripherin is a type III IF protein that has been shown to colocalise and interact with syncoilin. Our analyses suggest that syncoilin might function to modulate formation of peripherin filament networks through binding to peripherin isoforms. Peripherin is associated with the disease amyotrophic lateral sclerosis (ALS), thus establishing a link between syncoilin and ALS. A neuronal analysis of the syncoilin-null mouse (Sync(-/-)) revealed a reduced ability in accelerating treadmill and rotarod tests. This phenotype might be attributable to the impaired function of extensor digitorum longus muscle and type IIb fibres caused by a shift from large- to small-calibre motor axons in the ventral root.

Full Text

Duke Authors

Cited Authors

  • Clarke, WT; Edwards, B; McCullagh, KJA; Kemp, MW; Moorwood, C; Sherman, DL; Burgess, M; Davies, KE

Published Date

  • August 1, 2010

Published In

Volume / Issue

  • 123 / Pt 15

Start / End Page

  • 2543 - 2552

PubMed ID

  • 20587592

Pubmed Central ID

  • 20587592

Electronic International Standard Serial Number (EISSN)

  • 1477-9137

Digital Object Identifier (DOI)

  • 10.1242/jcs.059113

Language

  • eng

Conference Location

  • England