Journal
DEVELOPMENT
Volume 136, Issue 9, Pages 1571-1581Publisher
COMPANY BIOLOGISTS LTD
DOI: 10.1242/dev.029983
Keywords
Drosophila; HRD; Spastin; TBCE (CG7861); Tubulin chaperone
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Funding
- National Science Foundation of China (NSFC) [30871368]
- NSFC [30430250, 30525015]
- Ministry of Science and Technology of China [2006AA02Z166, 2007CB947200]
- Chinese Academy of Sciences [KSCX1-YW-R-69]
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Hypoparathyroidism, mental retardation and facial dysmorphism (HRD) is a fatal developmental disease caused by mutations in tubulin-specific chaperone E (TBCE). A mouse Tbce mutation causes progressive motor neuronopathy. To dissect the functions of TBCE and the pathogenesis of HRD, we generated mutations in Drosophila tbce, and manipulated its expression in a tissue-specific manner. Drosophila tbce nulls are embryonic lethal. Tissue-specific knockdown and overexpression of tbce in neuromusculature resulted in disrupted and increased microtubules, respectively. Alterations in TBCE expression also affected neuromuscular synapses. Genetic analyses revealed an antagonistic interaction between TBCE and the microtubule-severing protein Spastin. Moreover, treatment of muscles with the microtubule-depolymerizing drug nocodazole implicated TBCE as a tubulin polymerizing protein. Taken together, our results demonstrate that TBCE is required for the normal development and function of neuromuscular synapses and that it promotes microtubule formation. As defective microtubules are implicated in many neurological and developmental diseases, our work on TBCE may offer novel insights into their basis.
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