Abstract The importance of bile acid synthesis and metabolism to normal physiology and their role in pathophysiological states is well recognized. For such small and relatively simple molecules, bile acids…
Abstract The liver is the largest parenchymal organ in the body and has a complicated circulatory system, with dual afferent blood supply from the portal vein and hepatic artery. Although…
Abstract Non-alcoholic fatty liver disease (NAFLD) is now the most common cause of chronic liver disease in both adults and children worldwide [1]. Increasing industrialization and commercial globalization in Asia,…
Abstract Peroxisomes are ubiquitous subcellular organelles that are found in essentially all animal and plant cells with the exception of mature anucleated erythrocytes. They carry out many essential biochemical processes,…
Abstract Mitochondrial fatty acid β-oxidation (FAO) is an essential component of energy production and homeostasis in humans. During periods of limited glucose supply, FAO in the liver provides energy for…
Abstract Lysosomes are membrane-bound cellular organelles that contain multiple hydrolases needed for the digestion of various macromolecules including mucopolysaccharides, glycosphingolipids, cholesterol esters and oligosaccharides. Moreover, the lysosome is emerging as…
Abstract The urea cycle, first described in 1932 by Krebs and Henseleit, is the only metabolic pathway responsible for converting nitrogenous waste, produced by the breakdown of protein and other…
Abstract Hepatorenal tyrosinemia1 is a severe inborn error of metabolism that can affect numerous organs, particularly the liver, kidneys, and peripheral nerves. In the first accounts of patients with features…
Abstract Excess copper in the liver is toxic in humans and other mammals, and may lead to acute or chronic hepatitis, steatohepatitis, acute liver failure, cirrhosis and death. Of the…
Abstract Iron overload states can be classified as primary or secondary. There are many disorders that can lead to iron overload (Table 29.1) [1, 2]. This chapter focuses on hereditary…
