title: Cell biology of molybdenum in plants and humans.
abstract: The transition element molybdenum (Mo) needs to be complexed by a special cofactor in order to gain catalytic activity. With the exception of bacterial Mo-nitrogenase, where Mo is a constituent of the FeMo-cofactor, Mo is bound to a pterin, thus forming the molybdenum cofactor Moco, which in different variants is the active compound at the catalytic site of all other Mo-containing enzymes. In eukaryotes, the most prominent Mo-enzymes are nitrate reductase, sulfite oxidase, xanthine dehydrogenase, aldehyde oxidase, and the mitochondrial amidoxime reductase. The biosynthesis of Moco involves the complex interaction of six proteins and is a process of four steps, which also requires iron, ATP and copper. After its synthesis, Moco is distributed to the apoproteins of Mo-enzymes by Moco-carrier/binding proteins. A deficiency in the biosynthesis of Moco has lethal consequences for the respective organisms. In humans, Moco deficiency is a severe inherited inborn error in metabolism resulting in severe neurodegeneration in newborns and causing early childhood death. This article is part of a Special Issue entitled: Cell Biology of Metals.
authors.affiliation: Institute of Plant Biology, Braunschweig University of Technology, 1 Humboldt Street, 38106 Braunschweig, Germany. r.mendel@tu-bs.de
authors.forename: Ralf R
authors.initials: RR
authors.lastname: Mendel
authors.forename: Tobias
authors.initials: T
authors.lastname: Kruse
mesh_terms: D000255:Adenosine Triphosphate / Q000378:metabolism; D042931:Aldehyde Oxidase / Q000737:chemistry / Q000378:metabolism; D003067:Coenzymes / Q000737:chemistry / Q000378:metabolism; D003300:Copper / Q000378:metabolism; D020271:Heredodegenerative Disorders, Nervous System / Q000235:genetics* / Q000378:metabolism; D006801:Humans; D007231:Infant, Newborn; D007501:Iron / Q000378:metabolism; D008667:Metalloproteins / Q000737:chemistry / Q000378:metabolism; D008982:Molybdenum / Q000378:metabolism*; D000090006:Molybdenum Cofactors; D050901:Nitrate Reductase / Q000737:chemistry / Q000378:metabolism; D010088:Oxidoreductases / Q000737:chemistry / Q000378:metabolism; D010944:Plants / Q000378:metabolism*; D011621:Pteridines / Q000737:chemistry / Q000378:metabolism; D050876:Sulfite Oxidase / Q000737:chemistry / Q000378:metabolism; D013466:Sulfurtransferases / Q000172:deficiency / Q000235:genetics*; D014968:Xanthine Dehydrogenase / Q000737:chemistry / Q000378:metabolism
chemical_list: D000255:Adenosine Triphosphate; D042931:Aldehyde Oxidase; D003067:Coenzymes; D003300:Copper; D007501:Iron; D008667:Metalloproteins; D008982:Molybdenum; D000090006:Molybdenum Cofactors; D050901:Nitrate Reductase; D010088:Oxidoreductases; D011621:Pteridines; D050876:Sulfite Oxidase; D013466:Sulfurtransferases; D014968:Xanthine Dehydrogenase; C022261:molybdopterin; C534493:amidoxime reducing component, human; C425823:MOCOS protein, human