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- Scopus: eid_2-s2.0-42149158389
- PMID: 18366013
- WOS: WOS:000254993700011
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Article: Lamin A/C, laminopathies and premature ageing
Title | Lamin A/C, laminopathies and premature ageing |
---|---|
Authors | |
Keywords | Lamin A/C Laminopathy Premature ageing |
Issue Date | 2008 |
Publisher | Histology 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? |
Abstract | Lamin 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 Identifier | http://hdl.handle.net/10722/134120 |
ISSN | 2023 Impact Factor: 2.5 2023 SCImago Journal Rankings: 0.571 |
ISI Accession Number ID | |
References |
DC Field | Value | Language |
---|---|---|
dc.contributor.author | Liu, B | en_HK |
dc.contributor.author | Zhou, Z | en_HK |
dc.date.accessioned | 2011-06-13T07:19:28Z | - |
dc.date.available | 2011-06-13T07:19:28Z | - |
dc.date.issued | 2008 | en_HK |
dc.identifier.citation | Histology And Histopathology, 2008, v. 23 n. 6, p. 747-763 | en_HK |
dc.identifier.issn | 0213-3911 | en_HK |
dc.identifier.uri | http://hdl.handle.net/10722/134120 | - |
dc.description.abstract | Lamin 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.language | eng | en_US |
dc.publisher | Histology and Histopathology. The Journal's web site is located at http://www.hh.um.es | en_HK |
dc.relation.ispartof | Histology and Histopathology | en_HK |
dc.subject | Lamin A/C | en_HK |
dc.subject | Laminopathy | en_HK |
dc.subject | Premature ageing | en_HK |
dc.title | Lamin A/C, laminopathies and premature ageing | en_HK |
dc.type | Article | en_HK |
dc.identifier.email | Liu, B:ppliew@hkusua.hku.hk | en_HK |
dc.identifier.email | Zhou, Z:zhongjun@hkucc.hku.hk | en_HK |
dc.identifier.authority | Liu, B=rp01485 | en_HK |
dc.identifier.authority | Zhou, Z=rp00503 | en_HK |
dc.description.nature | link_to_subscribed_fulltext | en_US |
dc.identifier.pmid | 18366013 | - |
dc.identifier.scopus | eid_2-s2.0-42149158389 | en_HK |
dc.identifier.hkuros | 146097 | - |
dc.relation.references | http://www.scopus.com/mlt/select.url?eid=2-s2.0-42349117299&selection=ref&src=s&origin=recordpage | en_HK |
dc.identifier.volume | 23 | en_HK |
dc.identifier.issue | 6 | en_HK |
dc.identifier.spage | 747 | en_HK |
dc.identifier.epage | 763 | en_HK |
dc.identifier.isi | WOS:000254993700011 | - |
dc.publisher.place | Spain | en_HK |
dc.identifier.scopusauthorid | Liu, B=7408693394 | en_HK |
dc.identifier.scopusauthorid | Zhou, Z=8631856300 | en_HK |
dc.identifier.issnl | 0213-3911 | - |