Abstract:
:Dystrophin-deficiency causes cardiomyopathies and shortens the life expectancy of Duchenne and Becker muscular dystrophy patients. Restoring Dystrophin expression in the heart by gene transfer is a promising avenue to explore as a therapy. Truncated Dystrophin gene constructs have been engineered and shown to alleviate dystrophic skeletal muscle disease, but their potential in preventing the development of cardiomyopathy is not fully understood. In the present study, we found that either the mechanical or the signaling functions of Dystrophin were able to reduce the dilated heart phenotype of Dystrophin mutants in a Drosophila model. Our data suggest that Dystrophin retains some function in fly cardiomyocytes in the absence of a predicted mechanical link to the cytoskeleton. Interestingly, cardiac-specific manipulation of nitric oxide synthase expression also modulates cardiac function, which can in part be reversed by loss of Dystrophin function, further implying a signaling role of Dystrophin in the heart. These findings suggest that the signaling functions of Dystrophin protein are able to ameliorate the dilated cardiomyopathy, and thus might help to improve heart muscle function in micro-Dystrophin-based gene therapy approaches.
journal_name
Exp Gerontoljournal_title
Experimental gerontologyauthors
Taghli-Lamallem O,Jagla K,Chamberlain JS,Bodmer Rdoi
10.1016/j.exger.2013.10.015subject
Has Abstractpub_date
2014-01-01 00:00:00pages
26-34eissn
0531-5565issn
1873-6815pii
S0531-5565(13)00319-7journal_volume
49pub_type
杂志文章abstract::The accuracy of protein synthesis has been measured in extracts from human diploid fibroblasts of different ages. Extracts were supplied with purified mRNA for the coat protein of the cowpea variant of tobacco mosaic virus (CcTMV), which lacks codons for cysteine and methionine. The presence of 35S-cysteine in CcTMV c...
journal_title:Experimental gerontology
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doi:10.1016/0531-5565(89)90022-3
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journal_title:Experimental gerontology
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doi:10.1016/0531-5565(90)90020-3
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journal_title:Experimental gerontology
pub_type: 杂志文章
doi:10.1016/0531-5565(94)00053-0
更新日期:1995-03-01 00:00:00
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journal_title:Experimental gerontology
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abstract:BACKGROUND:Sarcopenic obesity is a combination of both sarcopenia and obesity, which potentiate each other and maximize the negative influences of each, such as physical disability, morbidity, or even mortality. OBJECTIVES:To describe the criteria used to identify people with sarcopenic obesity and the components of t...
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pub_type: 杂志文章,评审
doi:10.1016/j.exger.2020.110937
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abstract::A series of theoretical analyses of mortality were performed on recent data for Mediterranean fruit flies (Ceratitus capitata). Best fit computations were performed using the Marquardt-Levenburg technique on: 1) the classic Gompertz model, 2) the Johnson-Mehl-Avrami (JMA) model from chemical kinetics theory, which is ...
journal_title:Experimental gerontology
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journal_title:Experimental gerontology
pub_type: 杂志文章,评审
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journal_title:Experimental gerontology
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更新日期:2010-01-01 00:00:00
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journal_title:Experimental gerontology
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journal_title:Experimental gerontology
pub_type: 杂志文章
doi:10.1016/j.exger.2005.06.006
更新日期:2005-08-01 00:00:00
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journal_title:Experimental gerontology
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doi:10.1016/s0531-5565(01)00229-7
更新日期:2002-05-01 00:00:00
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journal_title:Experimental gerontology
pub_type: 杂志文章
doi:10.1016/s0531-5565(00)00125-x
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doi:10.1016/0531-5565(85)90038-5
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doi:10.1016/j.exger.2016.12.011
更新日期:2017-08-01 00:00:00
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journal_title:Experimental gerontology
pub_type: 杂志文章
doi:10.1016/j.exger.2018.11.005
更新日期:2019-03-01 00:00:00
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journal_title:Experimental gerontology
pub_type: 杂志文章
doi:10.1016/j.exger.2012.04.009
更新日期:2012-07-01 00:00:00
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journal_title:Experimental gerontology
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journal_title:Experimental gerontology
pub_type: 杂志文章
doi:10.1016/j.exger.2007.03.005
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journal_title:Experimental gerontology
pub_type: 杂志文章
doi:10.1016/0531-5565(89)90014-4
更新日期:1989-01-01 00:00:00
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journal_title:Experimental gerontology
pub_type: 杂志文章,多中心研究
doi:10.1016/j.exger.2015.05.012
更新日期:2015-10-01 00:00:00
abstract::Alzheimer's disease (AD) is the most common cause of dementia accounting for 60-70% of all demented cases and one of the leading sources of morbidity and mortality in the aging population. Most of the recent literature regards the relationship between plasma oxidative stress and AD, showing that markers of lipid perox...
journal_title:Experimental gerontology
pub_type: 杂志文章
doi:10.1016/j.exger.2019.110754
更新日期:2019-12-01 00:00:00
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journal_title:Experimental gerontology
pub_type: 评论,杂志文章
doi:10.1016/j.exger.2016.08.008
更新日期:2016-10-01 00:00:00
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journal_title:Experimental gerontology
pub_type: 杂志文章
doi:10.1016/s0531-5565(02)00087-6
更新日期:2002-08-01 00:00:00
abstract::The immune response declines with age. This decline correlates with thymic involution and involves primarily a loss in T-cell function, whereas humoral immunity is more variably affected. In the current experiments we have measured immunoglobulin synthesis in vitro after mitogen stimulation, and specific antibody resp...
journal_title:Experimental gerontology
pub_type: 杂志文章
doi:10.1016/0531-5565(86)90017-3
更新日期:1986-01-01 00:00:00
abstract::We have identified a multistep mechanism by which the DNA virus SV40 overcomes cellular senescence. Expression of SV40 T antigen is required for both transient extension of life span and unlimited life span or immortalization. These effects are mediated through inactivation of function of growth suppressors pRB and p5...
journal_title:Experimental gerontology
pub_type: 杂志文章,评审
doi:10.1016/0531-5565(95)00024-0
更新日期:1996-01-01 00:00:00
abstract::Plantar-flexor muscles are key muscles in the control of postural sway. Older fallers present lower maximal plantar-flexor performance than older non-fallers; however, the mechanisms underlying this motor impairment remain to be elucidated. This study aimed to determine whether muscular and neural factors are both inv...
journal_title:Experimental gerontology
pub_type: 杂志文章
doi:10.1016/j.exger.2018.09.011
更新日期:2018-10-02 00:00:00
abstract:OBJECTIVES:To date studies evaluating the relation between circulating aldosterone levels and mortality in elderly female individuals are lacking. We therefore aimed to assess the relationship between circulating aldosterone levels and mortality in a population-based cohort study of female nursing home residents. METH...
journal_title:Experimental gerontology
pub_type: 杂志文章,多中心研究
doi:10.1016/j.exger.2013.01.004
更新日期:2013-03-01 00:00:00