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首页> 外文期刊>The journals of gerontology.Series A. Biological sciences and medical sciences >Progeria of stem cells: stem cell exhaustion in Hutchinson-Gilford progeria syndrome.
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Progeria of stem cells: stem cell exhaustion in Hutchinson-Gilford progeria syndrome.

机译：干细胞早衰：Hutchinson-Gilford早衰综合征中的干细胞衰竭。

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Hutchinson-Gilford progeria syndrome (HGPS) is a rare, fatal genetic disorder that is characterized by segmental accelerated aging. The major causal mutation associated with HGPS triggers abnormal messenger RNA splicing of the lamin A gene leading to changes in the nuclear architecture. To date, two models have been proposed to explain how mutations in the lamin A gene could lead to HGPS, structural fragility and altered gene expression. We favor a compatible model that links HGPS to stem cell-driven tissue regeneration. In this model, nuclear fragility of lamin A-deficient cells increases apoptotic cell death to levels that exhaust tissues' ability for stem cell-driven regeneration. Tissue-specific differences in cell death or regenerative potential, or both, result in the tissue-specific segmental aging pattern seen in HGPS. We propose that the pattern of aging-related conditions present or absent in HGPS can provide insight into the genetic and environmental factors that contribute to normal aging.
机译：Hutchinson-Gilford早衰综合症（HGPS）是一种罕见的致命遗传性疾病，其特征是节段性加速衰老。与HGPS相关的主要因果突变会触发lamin A基因的异常信使RNA剪接，导致核结构发生变化。迄今为止，已经提出了两种模型来解释lamin A基因中的突变如何导致HGPS，结构脆弱性和基因表达改变。我们赞成将HGPS与干细胞驱动的组织再生联系起来的兼容模型。在这种模型中，核纤层蛋白A缺陷型细胞的核脆性使凋亡细胞死亡增加至耗尽组织干细胞驱动的再生能力的水平。细胞死亡或再生潜能或两者的组织特异性差异导致在HGPS中看到的组织特异性节段性衰老模式。我们建议，HGPS中存在或不存在的与衰老相关的状况的模式可以提供洞悉有助于正常衰老的遗传和环境因素的信息。

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