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Comment
. 2019 Jan 7;29(1):R28-R30.
doi: 10.1016/j.cub.2018.11.031.

Microtubules and Neurodegeneration: The Tubulin Code Sets the Rules of the Road

J Chloë Bulinski  1
Affiliations
Free article
Comment

Microtubules and Neurodegeneration: The Tubulin Code Sets the Rules of the Road

J Chloë Bulinski. Curr Biol. .
Free article

Abstract

Two recent papers demonstrate that the 'tubulin code' - the pattern of chemical modifications of tubulin along a microtubule - is disrupted upon deletion or mutation of an enzyme, called CCP1, that removes one of these modifications. Ablation of CCP1 interferes with mitochondrial transport and causes human neurodegenerative disease, which may be amenable to pharmacological therapies.

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  • Excessive tubulin polyglutamylation causes neurodegeneration and perturbs neuronal transport.
    Magiera MM, Bodakuntla S, Žiak J, Lacomme S, Marques Sousa P, Leboucher S, Hausrat TJ, Bosc C, Andrieux A, Kneussel M, Landry M, Calas A, Balastik M, Janke C. Magiera MM, et al. EMBO J. 2018 Dec 3;37(23):e100440. doi: 10.15252/embj.2018100440. Epub 2018 Nov 12. EMBO J. 2018. PMID: 30420556 Free PMC article.
  • Loss of tubulin deglutamylase CCP1 causes infantile-onset neurodegeneration.
    Shashi V, Magiera MM, Klein D, Zaki M, Schoch K, Rudnik-Schöneborn S, Norman A, Lopes Abath Neto O, Dusl M, Yuan X, Bartesaghi L, De Marco P, Alfares AA, Marom R, Arold ST, Guzmán-Vega FJ, Pena LD, Smith EC, Steinlin M, Babiker MO, Mohassel P, Foley AR, Donkervoort S, Kaur R, Ghosh PS, Stanley V, Musaev D, Nava C, Mignot C, Keren B, Scala M, Tassano E, Picco P, Doneda P, Fiorillo C, Issa MY, Alassiri A, Alahmad A, Gerard A, Liu P, Yang Y, Ertl-Wagner B, Kranz PG, Wentzensen IM, Stucka R, Stong N, Allen AS, Goldstein DB; Undiagnosed Diseases Network; Schoser B, Rösler KM, Alfadhel M, Capra V, Chrast R, Strom TM, Kamsteeg EJ, Bönnemann CG, Gleeson JG, Martini R, Janke C, Senderek J. Shashi V, et al. EMBO J. 2018 Dec 3;37(23):e100540. doi: 10.15252/embj.2018100540. Epub 2018 Nov 12. EMBO J. 2018. PMID: 30420557 Free PMC article.

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