The COPI vesicle complex binds and moves with survival motor neuron within axons

Cyril Jayakumar Peter, Matthew Evans, Venugopal Thayanithy, Naoko Taniguchi-Ishigaki, Ingolf Bach, Adrianne Kolpak, Gary J. Bassell, Wilfried Rossoll, Christian L. Lorson, Zheng Zheng Bao, Elliot J. Androphy

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Abstract

Spinal muscular atrophy (SMA), an inherited disease of motor neuron dysfunction, results from insufficient levels of the survival motor neuron (SMN) protein. Movement of the SMN protein as granules within cultured axons suggests that the pathogenesis of SMA may involve defects in neuronal transport, yet the nature of axon transport vesicles remains enigmatic. Here we show that SMN directly binds to the a-subunit of the coat protein I (COPI) vesicle coat protein. The α-COP protein co-immunoprecipitates with SMN, small nuclear ribonucleoprotein-associated assembly factors and β-actin mRNA. Although typically Golgi associated, in neuronal cells α-COP localizes to lamellipodia and growth cones and moves within the axon, with a subset of these granules traveling together with SMN. Depletion of α-COP resulted in mislocalization of SMN and actin at the leading edge at the lamellipodia. We propose that neurons utilize the Golgi-associated COPI vesicle to deliver cargoes necessary for motor neuron integrity and function.

Original languageEnglish (US)
Article numberddr046
Pages (from-to)1701-1711
Number of pages11
JournalHuman molecular genetics
Volume20
Issue number9
DOIs
StatePublished - May 2011

ASJC Scopus subject areas

  • Molecular Biology
  • Genetics
  • Genetics(clinical)

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    Peter, C. J., Evans, M., Thayanithy, V., Taniguchi-Ishigaki, N., Bach, I., Kolpak, A., Bassell, G. J., Rossoll, W., Lorson, C. L., Bao, Z. Z., & Androphy, E. J. (2011). The COPI vesicle complex binds and moves with survival motor neuron within axons. Human molecular genetics, 20(9), 1701-1711. [ddr046]. https://doi.org/10.1093/hmg/ddr046