THE PERICHROMATIN COMPARTMENT OF THE CELL NUCLEUS
E.G. Pershina,1 K.N. Morozova,1,2 E.V. Kiseleva 1
1 Institute of Cytology and Genetics, Novosibirsk, and 2 Novosibirsk State University;
e-mail: morozko@bionet.nsc.ru
This review describes the nesprins (nuclear envelope spectrin-repeat proteins), which are recently discovered
family of nuclear envelope proteins. These proteins play an important role in maintaining the cellular architecture
and establish the link between the nucleus and other sub-cellular compartments. Many tissue-specific
diseases including lipodystrophies, hearing loss, cardiac and skeletal myopathies are associated with nesprins
mutations. These proteins comprise of multiple tissue specific isoforms which contain spectrin repeats providing
interaction of nesprins with other nuclear membrane proteins, cytoskeleton and intranuclear matrix. We
summarize recent findings and suggestions about nesprins structural organization and function inside the cell.
Human diseases caused by abnormal nesprins expression are also described.
Key words: nesprins, nuclear envelope, laminopaties, cytoskeleton, nuclear matrics
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