Abnormal Golgi morphology and decreased COPI function in cells with low levels of SMN

S. K. Custer, J. N. Foster, J. W. Astroski, E. J. Androphy

Research output: Contribution to journalArticle

2 Scopus citations


We report here the finding of abnormal Golgi apparatus morphology in motor neuron like cells depleted of SMN as well as Golgi apparatus morphology in SMA patient fibroblasts. Rescue experiments demonstrate that this abnormality is dependent on SMN, but can also be rescued by expression of the COPI coatomer subunit alpha-COP. A motor neuron-like cell line containing an inducible alpha-COP shRNA was created to generate a parallel system to study knockdown of SMN or alpha-COP. Multiple assays of COPI-dependent intracellular trafficking in cells depleted of SMN demonstrate that alpha-COP function is suboptimal, including failed sequestration of plasma membrane proteins, altered binding of mRNA, and defective targeting and transport of Golgi-resident proteins.

Original languageEnglish (US)
Pages (from-to)135-146
Number of pages12
JournalBrain research
StatePublished - Mar 1 2019
Externally publishedYes


  • COPI
  • Coatomer
  • Golgi
  • Motor neuron disease
  • Spinal Muscular Atrophy
  • Survival motor neuron

ASJC Scopus subject areas

  • Neuroscience(all)
  • Molecular Biology
  • Clinical Neurology
  • Developmental Biology

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