Source:http://linkedlifedata.com/resource/pubmed/id/17725496
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Predicate | Object |
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rdf:type | |
lifeskim:mentions | |
pubmed:issue |
4
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pubmed:dateCreated |
2007-8-29
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pubmed:abstractText |
The precursor protein of the nuclear lamina constituent lamin A is a 74-kDa protein called prelamin A which undergoes subsequent steps of posttranslational modification at its C-terminal CaaX residue. The unexpected finding that accumulation of unprocessable prelamin A is the molecular basis of the most severe laminopathies so far identified, including Hutchinson-Gilford progeria and restrictive dermopathy, has opened new perspectives in the study of the pathogenic mechanisms causing all lamin A/C-linked disorders, as well as new interest in the analysis of molecular mechanisms regulating prelamin A processing. However, complete knowledge of the cellular pathways affected downstream of prelamin A accumulation is still lacking, but it could give new insights both in normal and pathogenic mechanisms regulated by lamins. In this article, we review the involvement of defects of prelamin A processing in the pathogenesis of a group of laminopathies. In particular, we discuss the possibility that mutations leading to accumulation of particular forms of prelamin A result in specific nuclear abnormalities and impairment of nuclear functions leading to cell senescence or altered metabolism.
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pubmed:language |
eng
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pubmed:journal | |
pubmed:citationSubset |
IM
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pubmed:chemical | |
pubmed:status |
MEDLINE
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pubmed:issn |
1045-4403
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pubmed:author | |
pubmed:issnType |
Print
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pubmed:volume |
17
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pubmed:owner |
NLM
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pubmed:authorsComplete |
Y
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pubmed:pagination |
317-34
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pubmed:meshHeading |
pubmed-meshheading:17725496-Animals,
pubmed-meshheading:17725496-Cell Nucleus,
pubmed-meshheading:17725496-Disease Models, Animal,
pubmed-meshheading:17725496-Laminin,
pubmed-meshheading:17725496-Mutation,
pubmed-meshheading:17725496-Nuclear Proteins,
pubmed-meshheading:17725496-Protein Precursors,
pubmed-meshheading:17725496-Protein Processing, Post-Translational
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pubmed:year |
2007
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pubmed:articleTitle |
Involvement of prelamin A in laminopathies.
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pubmed:affiliation |
Laboratory of Cell Biology, Istituto Ortopedico Rizzoli, Bologna, Italy. maraldi@area.bo.cnr.it
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pubmed:publicationType |
Journal Article,
Review,
Research Support, Non-U.S. Gov't
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