Vascular cell types in progeria: victims or villains?

IF 12.8 1区 医学 Q1 BIOCHEMISTRY & MOLECULAR BIOLOGY
Ignacio Benedicto, Magda R Hamczyk, Beatriz Dorado, Vicente Andrés
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引用次数: 0

Abstract

Hutchinson-Gilford progeria syndrome (HGPS) is an ultrarare genetic disease caused by progerin, a broadly expressed mutant variant of lamin A protein that accelerates aging and leads to premature death typically in adolescence. Progerin affects many organs and reproduces many characteristics of physiological aging, with the main cause of death in HGPS being atherosclerotic cardiovascular disease (CVD). Due to the rarity of HGPS, advances in understanding the disease and progress toward new therapeutic approaches are crucially dependent on preclinical models. We discuss recent research developments from a variety of HGPS experimental systems, with a special focus on in vivo studies of the role of vascular smooth muscle cells (VSMCs) and endothelial cells (ECs) that are key players in atherosclerosis.

早衰症的血管细胞类型:受害者还是恶棍?
哈钦森-吉尔福德早衰综合征(HGPS)是一种由早衰蛋白引起的罕见遗传疾病,早衰蛋白是一种广泛表达的层合蛋白a的突变变体,它加速衰老,通常在青春期导致过早死亡。早衰蛋白影响许多器官并再现生理性衰老的许多特征,HGPS的主要死亡原因是动脉粥样硬化性心血管疾病(CVD)。由于HGPS罕见,了解疾病的进展和新治疗方法的进展至关重要地依赖于临床前模型。我们讨论了各种HGPS实验系统的最新研究进展,特别关注血管平滑肌细胞(VSMCs)和内皮细胞(ECs)在动脉粥样硬化中的关键作用的体内研究。
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来源期刊
Trends in molecular medicine
Trends in molecular medicine 医学-生化与分子生物学
CiteScore
24.60
自引率
0.00%
发文量
142
审稿时长
6-12 weeks
期刊介绍: Trends in Molecular Medicine (TMM) aims to offer concise and contextualized perspectives on the latest research advancing biomedical science toward better diagnosis, treatment, and prevention of human diseases. It focuses on research at the intersection of basic biology and clinical research, covering new concepts in human biology and pathology with clear implications for diagnostics and therapy. TMM reviews bridge the gap between bench and bedside, discussing research from preclinical studies to patient-enrolled trials. The major themes include disease mechanisms, tools and technologies, diagnostics, and therapeutics, with a preference for articles relevant to multiple themes. TMM serves as a platform for discussion, pushing traditional boundaries and fostering collaboration between scientists and clinicians. The journal seeks to publish provocative and authoritative articles that are also accessible to a broad audience, inspiring new directions in molecular medicine to enhance human health.
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