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Genetics of molybdenum cofactor deficiency

Human Genetics, 2000
Molybdenum cofactor (MoCo) deficiency leads to a combined deficiency of the molybdoenzymes sulphite oxidase, xanthine dehydrogenase and aldehyde oxidase. Effective therapy is not available for this rare disease, which results in neonatal seizures and other neurological symptoms identical to those of sulphite oxidase deficiency.
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Chemistry and biology of the molybdenum cofactor

Biochemical Society Transactions, 1985
All molybdenum-containing enzymes other than nitrogenase carry out either oxidative hydroxylation or reductive dehydroxylation of their substrates as exemplified by the reactions catalyzed by sulfite oxidase and nitrate reductase respectively, shown below. The results of X-ray absorbance fine structure studies on sulfite oxidase, xanthine dehydrogenase
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Molybdenum Cofactor Biosynthesis

2016
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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Molybdenum Cofactor Disorders

2013
Molybdenum (Mo) cofactor deficiency (MoCD) is characterized by neonatal seizures, high-pitch crying, convulsions, and abnormal EEG and MRI findings accompanied by rapidly progressing neurodegeneration. In the absence of treatment, patients usually die within the first years of life and show no neurodevelopmental improvement.
Günter Schwarz, Alex Veldman
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The nature of molybdenum-cofactor.

Zhonghua Minguo wei sheng wu xue za zhi = Chinese journal of microbiology, 1978
In vitro assembly of Neurospora crassa NADPH-nitrate reductase (EC1.6.6.2) could be effected by combing the nitrate induced Neurospora crassa mutant nit-1 with the extract of any known molybdenum-containing enzyme. The process involves the participation of a molybdenum-cofactor contributed by the molybdenum-enzyme fraction.
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The biosynthesis of the molybdenum cofactors

2015
The 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 ...
Mendel, Ralf R.   +1 more
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The pterin of the molybdenum cofactor.

Federation proceedings, 1982
The molybdenum cofactor common to a variety of molybdoenzymes has been shown to contain a novel pterin. The pterin has been isolated from sulfite oxidase from several sources, xanthine-oxidizing enzymes from milk and chicken liver, and nitrate reductase of Chlorella vulgaris after denaturation of the proteins in the presence of I2. Investigation of the
K V, Rajagopalan   +2 more
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[Molybdenum cofactor deficiency].

Ryoikibetsu shokogun shirizu, 1998
T, Matsuishi   +3 more
  +6 more sources

Molybdenum Cofactor Disorders

2022
Guenter Schwarz, Bernd C. Schwahn
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