The proposed project is primarily aimed at synthesising a large variety of molybdenum complexes that mimic the natural molybdenum cofactor of the respective enzymes.
Consequently this project addresses the human iSOD by developing synthetic structural models for the molybdenum cofactor active site of sulfite oxidase, testing their potential for incorporation into the biotechnologically generated apo-enzyme and evaluating the activity of the resulting semi-synthetic enzymes thereby assessing their suitability as future iSOD treatments.
A congenital molybdenum cofactor deficiency disease, seen in infants, results in interference with the ability of the body to use molybdenum in enzymes.
Genetics of molybdenum cofactor deficiency, Human Genetics 106(2): 157-63.
cPMP replacement therapy molybdenum cofactor
Biopharmaceutical company Alexion Pharmaceuticals Inc (Nasdaq:ALXN) has purchased patents and assets from Orphatec Pharmaceuticals GmbH, a development-stage biotechnology company, related to an investigational therapy for patients with molybdenum cofactor
deficiency (MoCD) Type A.
The reason of paroxysm of tachycardia in the patient 4 was the neurodegenerative disease and the deficit of molybdenum cofactor.
Damage of the electrical conduction system of the heart in case of neurodegenerative disease like deficiency of molybdenum cofactor, is due to the accumulation of toxic sulfite.
The uric acid concentration is a useful diagnostic marker for deficiencies of phosphoribosyl pyrophosphate synthetase (PRPPS)  hypoxanthine guanine phosphoribosyl transferase (HGPRT), purine nucleoside phosphorylase (PNP), xanthine dehydrogenase (XDH), and molybdenum cofactor
Regulation of the chlA locus of Escherichia coli K12: Involvement of the molybdenum cofactor
Metabolic Replacement Therapy Designed to Prevent Severe Brain Damage, Early Mortality in Infants with Molybdenum Cofactor
Deficiency (MoCD) Type A
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.