血红素对于参与体内重要生理功能的多种蛋白质至关重要，例如氧运输、药物代谢、类固醇生物合成、信号转导、抗氧化防御和线粒体呼吸。然而，由于血红素铁具有促进细胞分子氧化的能力，游离血红素具有潜在的细胞毒性。肝脏在血红素代谢中发挥着核心作用，对血红素合成、血红素解毒和血红素铁的回收有显着贡献。相反，血红素生物合成途径中的酶缺陷会引发与肝损伤高度相关的多系统疾病（卟啉症）。此外，越来越多的证据表明血红素会导致炎症、代谢和恶性肝病的结果。在这篇综述中，我们总结了肝脏对血红素代谢的贡献以及血红素稳态失调与肝脏疾病的关系。版权所有 © 2024 Duarte、Viveiros、Godinho 和 Duarte。

Heme is essential for a variety of proteins involved in vital physiological functions in the body, such as oxygen transport, drug metabolism, biosynthesis of steroids, signal transduction, antioxidant defense and mitochondrial respiration. However, free heme is potentially cytotoxic due to the capacity of heme iron to promote the oxidation of cellular molecules. The liver plays a central role in heme metabolism by significantly contributing to heme synthesis, heme detoxification, and recycling of heme iron. Conversely, enzymatic defects in the heme biosynthetic pathway originate multisystemic diseases (porphyrias) that are highly associated with liver damage. In addition, there is growing evidence that heme contributes to the outcomes of inflammatory, metabolic and malignant liver diseases. In this review, we summarize the contribution of the liver to heme metabolism and the association of heme dyshomeostasis with liver disease.Copyright © 2024 Duarte, Viveiros, Godinho and Duarte.