期刊
DEVELOPMENTAL CELL
卷 35, 期 2, 页码 186-198出版社
CELL PRESS
DOI: 10.1016/j.devcel.2015.09.018
关键词
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资金
- INSERM
- CNRS
- Strasbourg University
- Agence Nationale de la Recherche [08-GENOPAT-005, 10-LABX- 0030-INRT]
- Association Francaise contre les Myopathies [15352]
- Myotubular Trust
Nucleus positioning is key for intracellular organization, cell differentiation, and organ development and is affected in many diseases, including myopathies due to alteration in amphiphysin-2 (BIN1). The actin and microtubule cytoskeletons are essential for nucleus positioning, but their crosstalk in this process is sparsely characterized. Here, we report that impairment of amphiphysin/BIN1 in Caenorhabditis elegans, mammalian cells, or muscles from patients with centronuclear myopathy alters nuclear position and shape. We show that AMPH-1/BIN1 binds to nesprin and actin, as well as to the microtubule-binding protein CLIP170 in both species. Expression of the microtubule-anchoring CAP-GLY domain of CLIP170 fused to the nuclear-envelope-anchoring KASH domain of nesprin rescues nuclear positioning defects of amph-1 mutants. Amphiphysins thus play a central role in linking the nuclear envelope with the actin and microtubule cytoskeletons. We propose that BIN1 has a direct and evolutionarily conserved role in nuclear positioning, altered in myopathies.
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