Loss of A-type lamins and genomic instability.
Cell Cycle
; 8(23): 3860-5, 2009 Dec.
Article
em En
| MEDLINE
| ID: mdl-19901537
Research performed in the last few years has revealed important roles for the spatial and temporal organization of the genome on genome function and integrity. A challenge in the field is to determine the molecular mechanisms involved in the organization of genome function. A-type lamins, key structural components of the nucleus, have been implicated in the maintenance of nuclear architecture and chromatin structure. Interestingly, alterations of A-type lamins lead to defects in DNA replication and repair as well as gene transcription and silencing. Elucidating the functions of these proteins is a topical subject since alterations of A-type lamins are associated with a variety of human diseases, ranging from muscular dystrophies and premature aging syndromes to cancer. Here, we discuss novels roles for A-type lamins in the maintenance of telomere structure, length and function as well as in the stabilization of a key DNA damage response factor. These studies support the notion that increased genomic instability due to defects in telomere biology and DNA repair contribute to the pathogenesis of lamin-related diseases.
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Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Lamina Tipo A
/
Instabilidade Genômica
Limite:
Humans
Idioma:
En
Ano de publicação:
2009
Tipo de documento:
Article