Hypoplastic Left Heart Syndrome: Signaling & Molecular Perspectives, and the Road Ahead.
Hypoplastic Left Heart Syndrome: Signaling & Molecular Perspectives, and the Road Ahead.
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DOI：
10.3390/ijms242015249
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发表时间：
2023-10-17
影响因子：
5.6
通讯作者：
Wu M
中科院分区：
文献类型：
--
作者：
Datta S;Cao W;Skillman M;Wu M
左心发育不全综合征（HLHS）是一种致命的先天性心脏病（CHD），全球每10万名新生儿中有8-25人患病。多年来，临床干预，主要是外科手术，大大提高了受影响受试者的预期寿命。然而，HLHS的病因学基础至今仍不清楚。基于现有的研究范式，HLHS表现出一个复杂的遗传和信号级联过程介导的多因素病因模式。本文综述了HLHS的表型、产前和产后风险以及驱动HLHS发病的信号和分子机制。这篇综述讨论了为解决现有科学差距而进行的研究的潜在局限性和未来前景。解释HLHS病因的机制研究将有可能阐明新的药物靶点，并在未来开发针对HLHS的治疗方案。
Hypoplastic left heart syndrome (HLHS) is a lethal congenital heart disease (CHD) affecting 8–25 per 100,000 neonates globally. Clinical interventions, primarily surgical, have improved the life expectancy of the affected subjects substantially over the years. However, the etiological basis of HLHS remains fundamentally unclear to this day. Based upon the existing paradigm of studies, HLHS exhibits a multifactorial mode of etiology mediated by a complicated course of genetic and signaling cascade. This review presents a detailed outline of the HLHS phenotype, the prenatal and postnatal risks, and the signaling and molecular mechanisms driving HLHS pathogenesis. The review discusses the potential limitations and future perspectives of studies that can be undertaken to address the existing scientific gap. Mechanistic studies to explain HLHS etiology will potentially elucidate novel druggable targets and empower the development of therapeutic regimens against HLHS in the future.
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影响因子：
37.3
作者：
Datta MW;Hernandez AM;Schlicht MJ;Kahler AJ;DeGueme AM;Dhir R;Shah RB;Farach-Carson C;Barrett A;Datta S
通讯作者：
Datta S
DOI：
10.1161/circoutcomes.115.001956
发表时间：
2015-07-01
影响因子：
6.9
作者：
Anderson, Jeffrey B.;Beekman, Robert H.;Lannon, Carole
通讯作者：
Lannon, Carole
影响因子：
2.4
作者：
通讯作者：
--
影响因子：
2.4
作者：
Belmont JW
通讯作者：
Belmont JW
影响因子：
2.7
作者：
Joo JH;Taxter TJ;Munguba GC;Kim YH;Dhaduvai K;Dunn NW;Degan WJ;Oh SP;Sugrue SP
通讯作者：
Sugrue SP