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[NESPRINS--nuclear envelope proteins ensuring integrity].
- Source :
-
Tsitologiia [Tsitologiia] 2014; Vol. 56 (7), pp. 467-79. - Publication Year :
- 2014
-
Abstract
- 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.
- Subjects :
- Animals
Cytoskeleton chemistry
Cytoskeleton metabolism
Cytosol metabolism
Gene Expression
Hearing Loss genetics
Hearing Loss metabolism
Hearing Loss pathology
Humans
Lipodystrophy genetics
Lipodystrophy metabolism
Lipodystrophy pathology
Membrane Proteins chemistry
Membrane Proteins genetics
Microfilament Proteins chemistry
Microfilament Proteins genetics
Muscular Dystrophy, Emery-Dreifuss genetics
Muscular Dystrophy, Emery-Dreifuss metabolism
Muscular Dystrophy, Emery-Dreifuss pathology
Mutation
Nuclear Envelope chemistry
Nuclear Proteins chemistry
Nuclear Proteins genetics
Protein Isoforms chemistry
Protein Isoforms genetics
Protein Isoforms metabolism
Membrane Proteins metabolism
Microfilament Proteins metabolism
Nuclear Envelope metabolism
Nuclear Proteins metabolism
Subjects
Details
- Language :
- Russian
- ISSN :
- 0041-3771
- Volume :
- 56
- Issue :
- 7
- Database :
- MEDLINE
- Journal :
- Tsitologiia
- Publication Type :
- Academic Journal
- Accession number :
- 25696990