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Cell Mol Life Sci. 2009 May;66(9):1518-33. doi: 10.1007/s00018-008-8713-y.

SUN-domain and KASH-domain proteins during development, meiosis and disease.

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1
Department of Genetics, Hebrew University of Jerusalem, Jerusalem, 91904, Israel.

Abstract

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.

PMID:
19125221
DOI:
10.1007/s00018-008-8713-y
[Indexed for MEDLINE]
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