ArticleEBioMedicine2017
ATP-Binding Cassette Transporter A1 Deficiency in Human Induced Pluripotent Stem Cell-Derived Hepatocytes Abrogates HDL Biogenesis and Enhances Triglyceride Secretion.
Article in EBioMedicine, 2017. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 14 papers.
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Who cites it
14 citing papers in PubMed, 27 citations in OpenAlex.
- Tangier Disease.Current atherosclerosis reports · 2026Review
- Lipid Dysregulation in Tangier Disease: A Case Series and Metabolic Characterization.The Journal of clinical endocrinology and metabolism · 2025Article
- Ethanol extract ofFrontiers in pharmacology · 2025Article
- ANGPTL3 (Angiopoietin-Like 3) Preferentially Resides on High-Density Lipoprotein in the Human Circulation, Affecting Its Activity.Journal of the American Heart Association · 2023Article
- From Cells to Organs: The Present and Future of Regenerative Medicine.Advances in experimental medicine and biology · 2022Review
- Current Diagnosis and Management of Tangier Disease.Journal of atherosclerosis and thrombosis · 2021Review
- Novel approaches to liver disease diagnosis and modeling.Translational gastroenterology and hepatology · 2021Review
- The impact of ATP-binding cassette transporters on metabolic diseases.Nutrition & metabolism · 2020Review
- iPSC-Derived Hepatocytes as a Platform for Disease Modeling and Drug Discovery.Frontiers in medicine · 2019Review
- New considerations for hiPSC-based models of neuropsychiatric disorders.Molecular psychiatry · 2019Review
- Genome Editing: The Recent History and Perspective in Cardiovascular Diseases.Journal of the American College of Cardiology · 2017Review
- Modeling Inborn Errors of Hepatic Metabolism Using Induced Pluripotent Stem Cells.Arteriosclerosis, thrombosis, and vascular biology · 2017Review
- Hepatic ABCA1 deficiency is associated with delayed apolipoprotein B secretory trafficking and augmented VLDL triglyceride secretion.Biochimica et biophysica acta. Molecular and cell biology of lipids · 2017Article
- The iPSC Awakens ANGPTL3 in Tangier Disease.EBioMedicine · 2017Article
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16 authors at 4 institutions in 1 country.
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Abstract
Despite the recognized role of the ATP-binding Cassette Transporter A1 (ABCA1) in high-density lipoprotein (HDL) metabolism, our understanding of ABCA1 deficiency in human hepatocytes is limited. To define the functional effects of human hepatocyte ABCA1 deficiency, we generated induced pluripotent stem cell (iPSC)-derived hepatocyte-like cells (HLCs) from Tangier disease (TD) and matched control subjects. Control HLCs exhibited robust cholesterol efflux to apolipoprotein A-I (apoA-I) and formed nascent HDL particles. ABCA1-deficient HLCs failed to mediate lipid efflux or nascent HDL formation, but had elevated triglyceride (TG) secretion. Global transcriptome analysis revealed significantly increased ANGPTL3 expression in ABCA1-deficient HLCs. Angiopoietin-related protein 3 (ANGPTL3) was enriched in plasma of TD relative to control subjects. These results highlight the required role of ABCA1 in cholesterol efflux and nascent HDL formation by hepatocytes. Furthermore, our results suggest that hepatic ABCA1 deficiency results in increased hepatic TG and ANGPTL3 secretion, potentially underlying the elevated plasma TG levels in TD patients.
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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.