The many functions of the microtubule cytoskeleton are essential for shaping the development and maintaining the operation of the nervous system. With the recent discovery of congenital neurological disorders that result from mutations in genes that encode different α- and β-tubulin isotypes (TUBA1A, TUBB2B, TUBA8 and TUBB3), scientists have a novel paradigm to assess how select perturbations in microtubule function affect a range of cellular processes in humans. Moreover, important phenotypic distinctions found among the syndromes suggest that different tubulin isotypes can be utilized for distinct cellular functions during nervous system development. In the present review, we discuss: (i) the spectrum of congenital nervous system diseases that result from mutations in tubulin and MAPs (microtubule-associated proteins); (ii) the known or putative roles of these proteins during nervous system development; (iii) how the findings collectively support the ‘multi-tubulin’ hypothesis, which postulates that different tubulin isotypes may be required for specialized microtubule functions.
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Review Article|
April 15 2010
Distinct α- and β-tubulin isotypes are required for the positioning, differentiation and survival of neurons: new support for the ‘multi-tubulin’ hypothesis
Max A. Tischfield;
Max A. Tischfield
1
*Department of Neurology and Ophthalmology, Manten Center for Orphan Disease Research, Children's Hospital Boston, Harvard Medical School, Boston, MA, U.S.A.
†Howard Hughes Medical Institute, Chevy Chase, MD, U.S.A.
1To whom correspondence should be addressed at the present address: Department of Molecular Biology and Genetics, Johns Hopkins University School of Medicine, 725 North Wolfe St., PCTB 804, Baltimore, MD 21205, U.S.A. (mtischf1@jhmi.edu).
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Elizabeth C. Engle
Elizabeth C. Engle
*Department of Neurology and Ophthalmology, Manten Center for Orphan Disease Research, Children's Hospital Boston, Harvard Medical School, Boston, MA, U.S.A.
†Howard Hughes Medical Institute, Chevy Chase, MD, U.S.A.
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Publisher: Portland Press Ltd
Received:
February 17 2010
Revision Received:
March 08 2010
Accepted:
March 10 2010
Online ISSN: 1573-4935
Print ISSN: 0144-8463
© The Authors Journal compilation © 2010 Biochemical Society
2010
Biosci Rep (2010) 30 (5): 319–330.
Article history
Received:
February 17 2010
Revision Received:
March 08 2010
Accepted:
March 10 2010
Citation
Max A. Tischfield, Elizabeth C. Engle; Distinct α- and β-tubulin isotypes are required for the positioning, differentiation and survival of neurons: new support for the ‘multi-tubulin’ hypothesis. Biosci Rep 1 October 2010; 30 (5): 319–330. doi: https://doi.org/10.1042/BSR20100025
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