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Cell Mol Life Sci ; 66(9): 1518-33, 2009 May.
Article in English | MEDLINE | ID: mdl-19125221

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.


Subject(s)
Cell Nucleus/metabolism , Lamins/physiology , Meiosis/physiology , Membrane Proteins/physiology , Amino Acid Motifs/physiology , Animals , Apoptosis/physiology , Centrosome/metabolism , Cerebellar Ataxia/genetics , Chromosomes/metabolism , Cytoskeleton/metabolism , Humans , Lamins/chemistry , Lamins/genetics , Membrane Proteins/chemistry , Membrane Proteins/genetics , Models, Biological , Muscular Dystrophy, Emery-Dreifuss/genetics
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