Syncoilin Modulates Peripherin Filament Networks and is Necessary for Large-calibre Motor Neurons

Overview

Journal J Cell Sci

Specialty Cell Biology

Date 2010 Jul 1

PMID 20587592

Citations 15

Authors

W Thomas Clarke

Ben Edwards

Karl J A McCullagh

Matthew W Kemp

Catherine Moorwood

Diane L Sherman

Matthew Burgess

Kay E Davies

Affiliations

Soon will be listed here.

Abstract

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.

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