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Article: Lamin A/C, laminopathies and premature ageing

TitleLamin A/C, laminopathies and premature ageing
Authors
KeywordsLamin A/C
Laminopathy
Premature ageing
Issue Date2008
PublisherHistology and Histopathology. The Journal's web site is located at http://www.hh.um.es
Citation
Histology And Histopathology, 2008, v. 23 n. 6, p. 747-763 How to Cite?
AbstractLamin A/C belongs to type V intermediate filaments and constitutes the nuclear lamina and nuclear matrix, where a variety of nuclear activities occur. Lamin A/C protein is firstly synthesized as a precursor and is further proteolytically processed by the zinc metallo-proteinase Ste24 (Zmpste24). Lamin A/C mutations cause a series of human diseases, collectively called laminopathies, the most severe of which is Hutchinson Gilford progeria syndrome (HGPS) and restrictive dermopathy (RD) which arises due to an unsuccessful maturation of prelamin A. Although the exact underlying molecular mechanisms are still poorly understood, genomic instability, defective nuclear mechanics and mechanotransduction, have been hypothesized to be responsible for laminopathy-based premature ageing. Removal of unprocessed prelamin A (progerin) or rescue of defective DNA repair could be potential therapeutic strategies for the treatment of HGPS in future.
Persistent Identifierhttp://hdl.handle.net/10722/134120
ISSN
2015 Impact Factor: 1.875
2015 SCImago Journal Rankings: 0.805
ISI Accession Number ID
References

 

DC FieldValueLanguage
dc.contributor.authorLiu, Ben_HK
dc.contributor.authorZhou, Zen_HK
dc.date.accessioned2011-06-13T07:19:28Z-
dc.date.available2011-06-13T07:19:28Z-
dc.date.issued2008en_HK
dc.identifier.citationHistology And Histopathology, 2008, v. 23 n. 6, p. 747-763en_HK
dc.identifier.issn0213-3911en_HK
dc.identifier.urihttp://hdl.handle.net/10722/134120-
dc.description.abstractLamin A/C belongs to type V intermediate filaments and constitutes the nuclear lamina and nuclear matrix, where a variety of nuclear activities occur. Lamin A/C protein is firstly synthesized as a precursor and is further proteolytically processed by the zinc metallo-proteinase Ste24 (Zmpste24). Lamin A/C mutations cause a series of human diseases, collectively called laminopathies, the most severe of which is Hutchinson Gilford progeria syndrome (HGPS) and restrictive dermopathy (RD) which arises due to an unsuccessful maturation of prelamin A. Although the exact underlying molecular mechanisms are still poorly understood, genomic instability, defective nuclear mechanics and mechanotransduction, have been hypothesized to be responsible for laminopathy-based premature ageing. Removal of unprocessed prelamin A (progerin) or rescue of defective DNA repair could be potential therapeutic strategies for the treatment of HGPS in future.en_HK
dc.languageengen_US
dc.publisherHistology and Histopathology. The Journal's web site is located at http://www.hh.um.esen_HK
dc.relation.ispartofHistology and Histopathologyen_HK
dc.subjectLamin A/Cen_HK
dc.subjectLaminopathyen_HK
dc.subjectPremature ageingen_HK
dc.titleLamin A/C, laminopathies and premature ageingen_HK
dc.typeArticleen_HK
dc.identifier.emailLiu, B:ppliew@hkusua.hku.hken_HK
dc.identifier.emailZhou, Z:zhongjun@hkucc.hku.hken_HK
dc.identifier.authorityLiu, B=rp01485en_HK
dc.identifier.authorityZhou, Z=rp00503en_HK
dc.description.naturelink_to_subscribed_fulltexten_US
dc.identifier.pmid18366013-
dc.identifier.scopuseid_2-s2.0-42149158389en_HK
dc.identifier.hkuros146097-
dc.relation.referenceshttp://www.scopus.com/mlt/select.url?eid=2-s2.0-42349117299&selection=ref&src=s&origin=recordpageen_HK
dc.identifier.volume23en_HK
dc.identifier.issue6en_HK
dc.identifier.spage747en_HK
dc.identifier.epage763en_HK
dc.identifier.isiWOS:000254993700011-
dc.publisher.placeSpainen_HK
dc.identifier.scopusauthoridLiu, B=7408693394en_HK
dc.identifier.scopusauthoridZhou, Z=8631856300en_HK

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