期刊
CELLULAR AND MOLECULAR LIFE SCIENCES
卷 66, 期 9, 页码 1518-1533出版社
SPRINGER BASEL AG
DOI: 10.1007/s00018-008-8713-y
关键词
Centrosome; cytoskeleton; Emery Dreifuss muscular dystrophy; homologous pairing; meiosis; nuclear positioning
资金
- Israel Ministry of Health
- USA-Israel Binational Science Foundation (BSF)
- Israel Science Foundation
- Muscular Dystrophy Association
- WWTF [LS05009]
- FWF
SUN-domain proteins interact directly with KASH-domain proteins to form protein complexes that connect the nucleus to every major cytoskeleton network. SUN-KASH protein complexes are also required for attaching centrosomes to the nuclear periphery and for alignment of homologous chromosomes, their pairing and recombination in meiosis. Other functions that require SUN-domain proteins include the regulation of apoptosis and maturation and survival of the germline. Laminopathic diseases affect the distribution of the SUN-KASH complexes, and mutations in KASH-domain proteins can cause Emery Dreifuss muscular dystrophy and recessive cerebellar ataxia. This review describes our current knowledge of the role of SUN-KASH domain protein complexes during development, meiosis and disease.
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