The liver has a pivotal role in the regulation of body homeostasis and hosts the majority of metabolic processes. Among these, the most relevant are glucose, lipid, amino acid and lipoprotein metabolisms, as well as bile formation, contributing to fat digestion and detoxification of xenobiotics. The majority of plasmatic proteins (albumin, acute-phase proteins, coagulation proteins) are synthesised in the hepatocytes, which represent 60–70% of all liver cells. The other 30–40% include endothelial cells, Kupffer cells and stellate cells. The hepatocytes are rich in mitochondria, double-membrane intracellular organelles containing a soluble matrix and their own unique genome, which are the main source of energy through the formation of ATP. The mitochondrial respiratory chain represents the ultimate step of energy production for all the main metabolic pathways. Since these organelles are highly concentrated in the hepatocytes, mitochondrial disorders are generally highly expressed in the liver. The functional development of the liver requires a complicated orchestration of changes in hepatic enzymes and metabolic pathways that result in the mature capacity to undertake metabolism, biotransformation and transport. The liver at birth has an overall immature metabolic function and achieves the adult expression of the drug-metabolizing enzymes CYP450 and UGT only after 1 year of age.
Casotti, V., D'Antiga, L. (2019). Principles of liver physiology. In L. D'Antiga (a cura di), Pediatric Hepatology and Liver Transplantation (pp. 21-39). Springer International Publishing [10.1007/978-3-319-96400-3_2].
Principles of liver physiology
D'antiga L.
2019
Abstract
The liver has a pivotal role in the regulation of body homeostasis and hosts the majority of metabolic processes. Among these, the most relevant are glucose, lipid, amino acid and lipoprotein metabolisms, as well as bile formation, contributing to fat digestion and detoxification of xenobiotics. The majority of plasmatic proteins (albumin, acute-phase proteins, coagulation proteins) are synthesised in the hepatocytes, which represent 60–70% of all liver cells. The other 30–40% include endothelial cells, Kupffer cells and stellate cells. The hepatocytes are rich in mitochondria, double-membrane intracellular organelles containing a soluble matrix and their own unique genome, which are the main source of energy through the formation of ATP. The mitochondrial respiratory chain represents the ultimate step of energy production for all the main metabolic pathways. Since these organelles are highly concentrated in the hepatocytes, mitochondrial disorders are generally highly expressed in the liver. The functional development of the liver requires a complicated orchestration of changes in hepatic enzymes and metabolic pathways that result in the mature capacity to undertake metabolism, biotransformation and transport. The liver at birth has an overall immature metabolic function and achieves the adult expression of the drug-metabolizing enzymes CYP450 and UGT only after 1 year of age.
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