Elongator - an emerging role in neurological disorders.
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.
Nguyen
Laurent
L
Interdisciplinary Cluster for Applied Genoproteomics (GIGA-Research), University of Liège, C.H.U. Sart Tilman, Liège, Belgium. lnguyen@ulg.ac.be
Humbert
Sandrine
S
Saudou
Frédéric
F
Chariot
Alain
A
D004402:Dysautonomia, Familial / Q000235:genetics / Q000378:metabolism; D051548:Histone Acetyltransferases / Q000235:genetics / Q000378:metabolism*; D006801:Humans; D008870:Microtubules / Q000235:genetics / Q000378:metabolism; D009419:Nerve Tissue Proteins / Q000235:genetics / Q000378:metabolism*; D009422:Nervous System Diseases / Q000235:genetics / Q000378:metabolism*; D009474:Neurons / Q000378:metabolism; D014404:Tubulin / Q000235:genetics / Q000378:metabolism
D009419:Nerve Tissue Proteins; D014404:Tubulin; C538729:ELP3 protein, human; D051548:Histone Acetyltransferases