molybdenum cofactor

mo·lyb·de·num co·fac·tor

a complex of molybdenum and molybdopterin required for a number of enzymes. A deficiency of this cofactor will result in lower activities of sulfite oxidase, xanthine dehydrogenase, and aldehyde oxidase causing elevated levels of sulfite, thiosulfite, xanthine, etc.
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References in periodicals archive ?
Keywords: Hypoxic-ischemic encephalopathy, Molybdenum cofactor deficiency, Pyruvate carboxylase deficiency, Zellweger syndrome, Non-ketotichyperglycinaemia.
The enzyme is used to treat patients with the ultra-rare disease caused by molybdenum cofactor deficiency Type A.
It was reported yesterday that the contract has been signed to acquire cyclic pyranopterin monophosphate (cPMP; ALXN1101), a synthetic enzyme co-factor therapy for patients with the ultra-rare disease caused by molybdenum cofactor deficiency (MoCD) Type A.
Prokaryotic nitrate reductases include a class of assimilatory enzymes and two classes of respiratory enzymes; all contain a guanylate molybdenum cofactor but differ in their substructures, cellular location, and requirement for cofactor.
A congenital molybdenum cofactor deficiency disease, seen in infants, results in interference with the ability of the body to use molybdenum in enzymes.
cPMP replacement therapy molybdenum cofactor deficiency
The reason of paroxysm of tachycardia in the patient 4 was the neurodegenerative disease and the deficit of molybdenum cofactor. The termination of pregnancy is the method of choice in full term newborns with paroxysms of tachycardia since the maturity of the lung parenchyma allows the initiation of the operation depending on the fetal condition.
The uric acid concentration is a useful diagnostic marker for deficiencies of phosphoribosyl pyrophosphate synthetase (PRPPS) [4] hypoxanthine guanine phosphoribosyl transferase (HGPRT), purine nucleoside phosphorylase (PNP), xanthine dehydrogenase (XDH), and molybdenum cofactor (2).
In the patients with inborn errors of purine metabolism, [sup.1]H-NMR spectroscopy of urine samples led to the diagnosis in all patients with PNP deficiency, xanthinuria, molybdenum cofactor deficiency, Lesch-Nyhan disease, ADA deficiency, and adenylosuccinate lyase deficiency.