Elongator - an emerging role in neurological disorders

Trends Mol Med. 2010 Jan;16(1):1-6. doi: 10.1016/j.molmed.2009.11.002. Epub 2009 Dec 23.


Neurological disorders are becoming a major public health issue in our aging society. An important objective is to understand the molecular events that underlie these diseases to prevent their onset and/or halt their progression. Acetylation of alpha-tubulin is a post-translational modification of microtubules that serves as a recognition signal for the anchoring of molecular motors and, as such, underlies the transport of various proteins or organelles in neurons. This process is affected in striatal and cortical neurons from Huntington's disease patients. Recent studies have shown that Elp3, the catalytic subunit of the Elongator complex, promotes the acetylation of alpha-tubulin in microtubules. Elongator complex activity is impaired in patients with familial dysautonomia. Based on converging experimental and clinical evidence, we propose that Elongator might be commonly targeted in different neurological disorders, and thus might represent a strong candidate for research and development efforts to design drug-based therapies.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Dysautonomia, Familial / genetics
  • Dysautonomia, Familial / metabolism
  • Histone Acetyltransferases / genetics
  • Histone Acetyltransferases / metabolism*
  • Humans
  • Microtubules / genetics
  • Microtubules / metabolism
  • Nerve Tissue Proteins / genetics
  • Nerve Tissue Proteins / metabolism*
  • Nervous System Diseases / genetics
  • Nervous System Diseases / metabolism*
  • Neurons / metabolism
  • Tubulin / genetics
  • Tubulin / metabolism


  • Nerve Tissue Proteins
  • Tubulin
  • ELP3 protein, human
  • Histone Acetyltransferases