Journal Article

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 and Elliot J. Androphy

in Human Molecular Genetics

Volume 20, issue 9, pages 1701-1711
Published in print May 2011 | ISSN: 0964-6906
Published online February 2011 | e-ISSN: 1460-2083 | DOI:
The COPI vesicle complex binds and moves with survival motor neuron within axons

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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 α-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.

Journal Article.  5388 words.  Illustrated.

Subjects: Genetics and Genomics

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