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Molybdenum cofactors, enzymes and pathways
Nature, 2009The trace element molybdenum is essential for nearly all organisms and forms the catalytic centre of a large variety of enzymes such as nitrogenase, nitrate reductases, sulphite oxidase and xanthine oxidoreductases. Nature has developed two scaffolds holding molybdenum in place, the iron-molybdenum cofactor and pterin-based molybdenum cofactors ...
Guenter Schwarz +2 more
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Molybdenum Cofactor Biosynthesis
The biosynthesis of the molybdenum cofactor (Moco) is highly conserved among all kingdoms of life. In all molybdoenzymes with the exception of nitrogenase, the molybdenum atom is coordinated to a dithiolene group present in the pterin-based 6-alkyl side chain of molybdopterin (MPT).
Leimkühler, Silke (Prof. Dr.) +1 more
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Regulation of molybdenum cofactor of maize leaf
Molybdenum cofactor and nitrate reductase were extracted from maize leaf and assayed using established methods. While both applied nitrate and light increased nitrate reductase activity, only nitrate was required to increase molybdenum cofactor to its ...
Wilbur H. Campbell
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Pterin chemistry and its relationship to the molybdenum cofactor [PDF]
The molybdenum cofactor is composed of a molybdenum coordinated by one or two rather complicated ligands known as either molybdopterin or pyranopterin. Pterin is one of a large family of bicyclic N-heterocycles called pteridines.
Partha Basu, Sharon J N Burgmayer
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The biosynthesis of the molybdenum cofactor in bacteria is described with a detailed analysis of each individual reaction leading to the formation of stable intermediates during the synthesis of molybdopterin from GTP.
Silke Leimkühler +2 more
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A Turkish Case with Molybdenum Cofactor Deficiency
Molybdenum cofactor deficiency (MIM 252150) is a rare progressive neurodegenerative disorder with about 100 cases reported worldwide. We have identified a male with molybdenum cofactor deficiency and analyzed the molybdenum cofactor synthesis (MOCS) 1 ...
Ali Dursun +2 more
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Molybdenum cofactor deficiency
The Journal of Pediatrics, 1993We describe a new case of molybdenum cofactor deficiency, an underrecognized inborn error of metabolism that results in neonatal seizures and neurologic abnormalities. Characteristic biochemical defects in affected individuals include hypouricemia, elevated urine sulfate (detectable by dipstick), and elevated S-sulfocysteine (detectable by anion ...
G L, Arnold +3 more
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Molybdenum cofactor deficiency
Molecular Genetics and Metabolism, 2016Molybdenum cofactor deficiency (MoCD) is a severe autosomal recessive inborn error of metabolism first described in 1978. It is characterized by a neonatal presentation of intractable seizures, feeding difficulties, severe developmental delay, microcephaly with brain atrophy and coarse facial features.
Paldeep S, Atwal, Fernando, Scaglia
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The molybdenum cofactor is an important cofactor, and its biosynthesis is essential for many organisms, including humans. Its basic form comprises a single molybdopterin (MPT) unit, which binds a molybdenum ion bearing three oxygen ligands via a ...
Michael Haumann, Kajsa G V Sigfridsson
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The biosynthesis of the molybdenum cofactors
JBIC Journal of Biological Inorganic Chemistry, 2014The biosynthesis of the molybdenum cofactors (Moco) is an ancient, ubiquitous, and highly conserved pathway leading to the biochemical activation of molybdenum. Moco is the essential component of a group of redox enzymes, which are diverse in terms of their phylogenetic distribution and their architectures, both at the overall level and in their ...
Ralf R, Mendel, Silke, Leimkühler
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