Perez-Luz Sara, Matamala Nerea, Gomez-Mariano Gema, Janciauskiene Sabina, Martínez-Delgado Beatriz
Molecular Genetics Unit, Institute of Rare Diseases Research (IIER), Institute of Health Carlos III (ISCIII), 28220 Madrid, Spain.
Department of Respiratory Medicine and Infectious Diseases, Biomedical Research in Endstage and Obstructive Lung Disease Hannover BREATH, Member of the German Center for Lung Research DZL, Hannover Medical School, 30625 Hannover, Germany.
Biomedicines. 2023 Jul 12;11(7):1961. doi: 10.3390/biomedicines11071961.
Non-alcoholic fatty liver disease (NAFLD) is a type of steatosis commonly associated with obesity, dyslipidemia, hypertension, and diabetes. Other diseases such as inherited alpha-1 antitrypsin deficiency (AATD) have also been related to the development of liver steatosis. The primary reasons leading to hepatic lipid deposits can be genetic and epigenetic, and the outcomes range from benign steatosis to liver failure, as well as to extrahepatic diseases. Progressive hepatocellular damage and dysregulated systemic immune responses can affect extrahepatic organs, specifically the heart and lungs. In this review, we discuss the similarities and differences between the molecular pathways of NAFLD and AATD, and the putative value of hepatic organoids as novel models to investigate the physio pathological mechanisms of liver steatosis.
非酒精性脂肪性肝病（NAFLD）是一种通常与肥胖、血脂异常、高血压和糖尿病相关的脂肪变性。其他疾病，如遗传性α-1抗胰蛋白酶缺乏症（AATD）也与肝脂肪变性的发展有关。导致肝脏脂质沉积的主要原因可能是遗传和表观遗传的，其结果范围从良性脂肪变性到肝衰竭，以及肝外疾病。进行性肝细胞损伤和失调的全身免疫反应可影响肝外器官，特别是心脏和肺。在这篇综述中，我们讨论了NAFLD和AATD分子途径之间的异同，以及肝类器官作为研究肝脂肪变性生理病理机制的新型模型的潜在价值。
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