This chapter reviews the two mitochondrial flavin containing isozymes of monoamine oxidase. Section 1, "Biochemistry" discusses assays, substrates and inhibitors, phylogenic and tissue distribution, interactions with lipids, nutritional studies, protein structure, kinetic and chemical mechanistic proposals, and biosynthesis. Section 2, "Inheritance" discusses possible genes involved in expression, genetic studies of platelet MAO-B and fibroblast MAO-A, and chromosomal location. Section 3, "Molecular Genetics" reviews the cloning of their cDNAs, their intra- and interspecies homology and structural inferences made from deduced amino acid sequences. Section 4, "Regulation" gives an overview of levels in development and aging, and effect of drugs. The final section 5, "Role in Human Disease" discusses physiological function and effects of altered levels in humans and animal models including complete absence due to a submicroscopic chromosomal deletion in several human patients.