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

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, b...

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Main Authors: Clarke, W, Edwards, B, McCullagh, K, Kemp, M, Moorwood, C, Sherman, D, Burgess, M, Davies, K
Format: Journal article
Language:English
Published: 2010
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author Clarke, W
Edwards, B
McCullagh, K
Kemp, M
Moorwood, C
Sherman, D
Burgess, M
Davies, K
author_facet Clarke, W
Edwards, B
McCullagh, K
Kemp, M
Moorwood, C
Sherman, D
Burgess, M
Davies, K
author_sort Clarke, W
collection OXFORD
description 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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spelling oxford-uuid:177df95f-a441-41cf-9d9f-05e4021b82a42022-03-26T10:37:36ZSyncoilin modulates peripherin filament networks and is necessary for large-calibre motor neurons.Journal articlehttp://purl.org/coar/resource_type/c_dcae04bcuuid:177df95f-a441-41cf-9d9f-05e4021b82a4EnglishSymplectic Elements at Oxford2010Clarke, WEdwards, BMcCullagh, KKemp, MMoorwood, CSherman, DBurgess, MDavies, KSyncoilin 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.
spellingShingle Clarke, W
Edwards, B
McCullagh, K
Kemp, M
Moorwood, C
Sherman, D
Burgess, M
Davies, K
Syncoilin modulates peripherin filament networks and is necessary for large-calibre motor neurons.
title Syncoilin modulates peripherin filament networks and is necessary for large-calibre motor neurons.
title_full Syncoilin modulates peripherin filament networks and is necessary for large-calibre motor neurons.
title_fullStr Syncoilin modulates peripherin filament networks and is necessary for large-calibre motor neurons.
title_full_unstemmed Syncoilin modulates peripherin filament networks and is necessary for large-calibre motor neurons.
title_short Syncoilin modulates peripherin filament networks and is necessary for large-calibre motor neurons.
title_sort syncoilin modulates peripherin filament networks and is necessary for large calibre motor neurons
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