molybdopterin (molybdenum cofactor)

also see records for molybdopterin cytosine dinucleotide and molybdopterin guanine dinucleotide

Also Known As:

molybdenum cofactor; 4H-pyrano(3,2-G)pteridin-4-one, 2-amino-3,5,5A,8,9A,10-hexahydro-6,7-dimercapto-8-((phosphonooxy)methyl)-, (5AR,8R,9AR)-; Moco cofactor; molybdenum cofactor precursor; molybdopterin cofactor; pterin molybdenum cofactor

Networked: 62 relevant articles (0 outcomes, 0 trials/studies)

Bio-Agent Context: Research Results

Enzymes and Coenzymes: 1
- Coenzymes: 558
  - Molybdenum Cofactors
    - molybdopterin: 62

Experts

1.	Reiss, Jochen: 5 articles (01/2016 - 12/2002)
2.	Kozmin, Stanislav G: 3 articles (10/2013 - 06/2007)
3.	Schaaper, Roel M: 3 articles (10/2013 - 06/2007)
4.	Schwarz, Günter: 3 articles (01/2011 - 12/2002)
5.	Santamaria-Araujo, José Angel: 2 articles (01/2022 - 06/2004)
6.	Schwarz, Guenter: 2 articles (01/2022 - 01/2016)
7.	Ichida, Kimiyoshi: 2 articles (03/2021 - 11/2012)
8.	Sass, Jörn Oliver: 2 articles (08/2010 - 06/2004)
9.	Hosoya, T: 2 articles (01/2006 - 04/2001)
10.	Ichida, K: 2 articles (01/2006 - 04/2001)

Related Diseases

1.	Molybdenum cofactor deficiency 01/01/2023 - "Molybdenum cofactor deficiency (MoCD) (OMIM# 252150) is an autosomal-recessive disorder caused by mutations in four genes involved in the molybdenum cofactor (MOCO) biosynthesis pathway. " 03/01/2021 - "Molybdenum cofactor deficiency (MoCD) is an autosomal recessive inborn error of metabolism that results from mutations in genes involved in molybdenum cofactor (Moco) biosynthesis. " 12/01/2006 - "The absence of TorD leads to a complete loss of apoTorA during molybdenum cofactor deficiency whereas the presence of TorD maintains a significant amount of apoTorA that can be matured when the molybdenum cofactor becomes available." 01/01/2006 - "We have identified a male with molybdenum cofactor deficiency and analyzed the molybdenum cofactor synthesis (MOCS)1 gene, MOCS2 gene, MOCS3 gene and GEPH gene. " 07/15/2000 - "Insights into molybdenum cofactor deficiency provided by the crystal structure of the molybdenum cofactor biosynthesis protein MoaC."
2.	Sulfite oxidase deficiency 01/01/2022 - "A defect in molybdenum cofactor binding causes an attenuated form of sulfite oxidase deficiency." 08/01/2010 - "We report clinical, radiologic, biochemical and genetic data on a Brazilian and on a Turkish child with sulfite oxidase deficiency due to the isolated defect and impaired synthesis of the molybdenum cofactor, respectively. " 10/01/2023 - "Sulfite oxidase (SO) deficiency, an inherited disease that causes severe neonatal neurological problems and early death, arises from defects in the biosynthesis of the molybdenum cofactor (Moco) (general sulfite oxidase deficiency) or from inborn errors in the SUOX gene for SO (isolated sulfite oxidase deficiency, ISOD). " 01/01/2017 - "Lastly, this assay is sensitive to detect reduced total homocysteine levels that are possibly seen in sulfocysteinuria and molybdenum cofactor deficiencies." 07/15/2005 - "Total truncation of the molybdopterin/dimerization domains of SUOX protein in an Arab family with isolated sulfite oxidase deficiency."
3.	Seizures (Absence Seizure) 01/01/2021 - "Molybdenum cofactor deficiencies should be considered in neonates with early-onset seizures, hypotonia, and feeding difficulties. " 12/01/2010 - "Molybdenum cofactor (Moco) deficiency is a rare neurometabolic disorder, characterized by neurological impairment and refractive seizures, due to toxic accumulation of sulfite in the brain. " 12/01/1998 - "Molybdenum cofactor (MoCo) deficiency is a rare and devastating disease resulting in neonatal seizures and other neurological symptoms identical to those of sulphite oxidase deficiency. " 12/01/2012 - "Based upon the clinically evident pyridoxine-responsive seizures in these 2 siblings, we recommend considering pyridoxine supplementation to patients affected with molybdenum cofactor or sulfite oxidase deficiencies." 12/15/2002 - "The absence of active sulfite oxidase-a molybdenum cofactor-dependent enzyme-results in neonatal seizures and early childhood death. "
4.	Type II Xanthinuria 11/01/2003 - "Identification of a new point mutation in the human molybdenum cofactor sulferase gene that is responsible for xanthinuria type II." 07/21/2000 - "Deletion mutation in Drosophila ma-l homologous, putative molybdopterin cofactor sulfurase gene is associated with bovine xanthinuria type II." 01/22/2002 - "The identification of this domain may clarify the mechanism of biogenesis of various metallo-enzymes including Molybdenum cofactor-containing enzymes that are compromised in human type II xanthinuria." 04/20/2001 - "These results indicate that a functional defect of the HMCS gene is responsible for classical xanthinuria type II, and that HMCS protein functions to provide a sulfur atom for the molybdenum cofactor of XDH and AO." 11/21/2012 - "Xanthinuria is classified into two subtypes, type I and type II. Type I xanthinuria involves XOR deficiency due to genetic defect of XOR, whereas type II xanthinuria involves dual deficiency of XOR and aldehyde oxidase (AO, a molybdoflavo enzyme similar to XOR) due to genetic defect in the molybdenum cofactor sulfurase. "
5.	Hypersensitivity (Allergy) 06/01/2007 - "Lack of molybdenum cofactor (MoCo) in Escherichia coli and related microorganisms was found to cause hypersensitivity to certain N-hydroxylated base analogs, such as HAP (6-N-hydroxylaminopurine). " 06/01/2000 - "Hypersensitivity of Escherichia coli Delta(uvrB-bio) mutants to 6-hydroxylaminopurine and other base analogs is due to a defect in molybdenum cofactor biosynthesis." 10/01/2013 - "Lack of molybdenum cofactor (Moco) in Escherichia coli leads to hypersensitivity to the mutagenic and toxic effects of N-hydroxylated base analogs, such as 6-N-hydroxylaminopurine (HAP). " 04/01/2008 - "We have shown previously that lack of molybdenum cofactor (MoCo) in Escherichia coli leads to hypersensitivity to the mutagenic and toxic effects of N-hydroxylated base analogues, such as 6-N-hydroxylaminopurine (HAP). "

Related Drugs and Biologics

1.	Sulfite Oxidase
2.	Enzymes
3.	Proteins (Proteins, Gene)
4.	Sulfites
5.	Aldehyde Oxidase
6.	Xanthine Dehydrogenase
7.	Xanthine Oxidase
8.	Sulfur
9.	Cysteine (L-Cysteine)
10.	6-N-hydroxylaminopurine

Related Therapies and Procedures

1.	Therapeutics