دورية أكاديمية
أعرض في EDS

Molecular Pathways and Animal Models of Hypoplastic Left Heart Syndrome.

التفاصيل البيبلوغرافية
العنوان: Molecular Pathways and Animal Models of Hypoplastic Left Heart Syndrome.
المؤلفون: Yagi H; Department of Developmental Biology, University of Pittsburgh School of Medicine, Pittsburgh, PA, USA., Xu X; Department of Developmental Biology, University of Pittsburgh School of Medicine, Pittsburgh, PA, USA., Gabriel GC; Department of Developmental Biology, University of Pittsburgh School of Medicine, Pittsburgh, PA, USA., Lo C; Department of Developmental Biology, University of Pittsburgh School of Medicine, Pittsburgh, PA, USA. cel36@pitt.edu.
المصدر: Advances in experimental medicine and biology [Adv Exp Med Biol] 2024; Vol. 1441, pp. 947-961.
نوع المنشور: Journal Article; Review
اللغة: English
بيانات الدورية: Publisher: Kluwer Academic/Plenum Publishers Country of Publication: United States NLM ID: 0121103 Publication Model: Print Cited Medium: Print ISSN: 0065-2598 (Print) Linking ISSN: 00652598 NLM ISO Abbreviation: Adv Exp Med Biol Subsets: MEDLINE
أسماء مطبوعة: Publication: 1998- : New York : Kluwer Academic/Plenum Publishers
Original Publication: New York, Plenum Press.
مواضيع طبية MeSH: Hypoplastic Left Heart Syndrome*/genetics , Hypoplastic Left Heart Syndrome*/pathology , Hypoplastic Left Heart Syndrome*/metabolism , Hypoplastic Left Heart Syndrome*/physiopathology , Disease Models, Animal* , Signal Transduction*, Animals ; Humans ; Mice ; Myocytes, Cardiac/metabolism ; Myocytes, Cardiac/pathology ; Induced Pluripotent Stem Cells/metabolism
مستخلص: Hypoplastic left heart syndrome (HLHS) is a severe congenital heart disease (CHD) with underdevelopment of left-sided heart structures. While previously uniformly fatal, surgical advances now provide highly effective palliation that allows most HLHS patients to survive their critical CHD. Nevertheless, there remains high morbidity and mortality with high risk of heart failure. As hemodynamic compromise from restricted aortic blood flow has been suggested to underlie the poor LV growth, this suggests the possibility of prenatal fetal intervention to recover LV growth. As such interventions have yielded ambiguous results, the optimization of therapy will require more mechanistic insights into the developmental etiology for HLHS. Clinical studies have shown high heritability for HLHS, with an oligogenic etiology indicated in conjunction with genetic heterogeneity. This is corroborated with the recent recovery of mutant mice with HLHS. With availability-induced pluripotent stem cell (iPSC)-derived cardiomyocytes from HLHS mice and patients, new insights have emerged into the cellular and molecular etiology for the LV hypoplasia in HLHS. Cell proliferation defects were observed in conjunction with metaphase arrest and the disturbance of Hippo-YAP signaling. The left-sided restriction of the ventricular hypoplasia may result from epigenetic perturbation of pathways regulating left-right patterning. These findings suggest new avenues for fetal interventions with therapies using existing drugs that target the Hippo-YAP pathway and/or modulate epigenetic regulation.
(© 2024. The Author(s), under exclusive license to Springer Nature Switzerland AG.)
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فهرسة مساهمة: Keywords: Congenital heart disease (CHD); Hippo signaling; Hypoplastic left heart syndrome (HLHS); Induced pluripotent stem cell-derived cardiomyocytes (iPSC-CM); Left ventricle (LV); Left ventricular outflow tract obstruction (LVOTO)
تواريخ الأحداث: Date Created: 20240617 Date Completed: 20240617 Latest Revision: 20240617
رمز التحديث: 20240617
DOI: 10.1007/978-3-031-44087-8_61
PMID: 38884763
قاعدة البيانات: MEDLINE
الوصف
تدمد:0065-2598
DOI:10.1007/978-3-031-44087-8_61