From lamins to lamina: a structural perspective.
Histochem Cell Biol
; 140(1): 3-12, 2013 Jul.
Article
em En
| MEDLINE
| ID: mdl-23744527
ABSTRACT
Lamin proteins are the major constituents of the nuclear lamina, a proteinaceous network that lines the inner nuclear membrane. Primarily, the nuclear lamina provides structural support for the nucleus and the nuclear envelope; however, lamins and their associated proteins are also involved in most of the nuclear processes, including DNA replication and repair, regulation of gene expression, and signaling. Mutations in human lamin A and associated proteins were found to cause a large number of diseases, termed 'laminopathies.' These diseases include muscular dystrophies, lipodystrophies, neuropathies, and premature aging syndromes. Despite the growing number of studies on lamins and their associated proteins, the molecular organization of lamins in health and disease is still elusive. Likewise, there is no comprehensive view how mutations in lamins result in a plethora of diseases, selectively affecting different tissues. Here, we discuss some of the structural aspects of lamins and the nuclear lamina organization, in light of recent results.
Texto completo:
1
Bases de dados:
MEDLINE
Assunto principal:
Lâmina Nuclear
/
Laminas
Limite:
Humans
Idioma:
En
Revista:
Histochem Cell Biol
Assunto da revista:
CITOLOGIA
/
HISTOCITOQUIMICA
Ano de publicação:
2013
Tipo de documento:
Article
País de afiliação:
Suíça