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.
Keywords. Centrosome, cytoskeleton, Emery Dreifuss muscular dystrophy, homologous pairing, meiosis, nuclear positioning
Footnotes
All authors contributed equally to this manuscript.
Received 07 November 2008; received after revision 07 December 2008; accepted 09 December 2008
Contributor Information
V. Jantsch, FAX: +43-1-4277 9562, Email: verena.jantsch@univie.ac.at
Y. Gruenbaum, FAX: +972-2-6586975, Email: gru@vms.huji.ac.il