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

J Cell Sci. 2010 Aug 1;123(Pt 15):2543-52. doi: 10.1242/jcs.059113. Epub 2010 Jun 29.


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.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Amyotrophic Lateral Sclerosis / etiology
  • Amyotrophic Lateral Sclerosis / genetics
  • Animals
  • Brain / metabolism
  • Cell Line, Tumor
  • Fluorescent Antibody Technique
  • Humans
  • Immunoblotting
  • Immunoprecipitation
  • Intermediate Filament Proteins / genetics
  • Intermediate Filament Proteins / metabolism*
  • Membrane Glycoproteins / genetics
  • Membrane Glycoproteins / metabolism*
  • Mice
  • Mice, Knockout
  • Motor Neurons / metabolism*
  • Muscle Proteins / genetics
  • Muscle Proteins / metabolism*
  • Nerve Tissue Proteins / genetics
  • Nerve Tissue Proteins / metabolism*
  • Peripherins
  • Polymerase Chain Reaction
  • Protein Binding
  • Protein Isoforms / genetics
  • Protein Isoforms / metabolism*
  • Sciatic Nerve / metabolism
  • Spinal Cord / metabolism


  • Intermediate Filament Proteins
  • Membrane Glycoproteins
  • Muscle Proteins
  • Nerve Tissue Proteins
  • PRPH protein, human
  • Peripherins
  • Protein Isoforms
  • SYNC protein, human
  • Sync protein, mouse