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Molybdenum cofactor and human disease
Abstract
Four molybdenum-dependent enzymes are known in humans, each harboring a pterin-based molybdenum cofactor (Moco) in the active site. They catalyze redox reactions using water as oxygen acceptor or donator. Moco is synthesized by a conserved biosynthetic pathway. Moco deficiency results in a severe inborn error of metabolism causing often early childhood death. Disease-causing symptoms mainly go back to the lack of sulfite oxidase (SO) activity, an enzyme in cysteine catabolism. Besides their name-giving functions, Mo-enzymes have been recognized to catalyze novel reactions, including the reduction of nitrite to nitric oxide. In this review we cover the biosynthesis of Moco, key features of Moco-enzymes and focus on their deficiency. Underlying disease mechanisms as well as treatment options will be discussed.
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“Febuxostat was approved in 2008 in Europe and by the Food and Drug Administration in 2009 for the treatment of gout (commercial name Uloric in United States, Adenuric in Europe) (76, 77, 837).”
From Sources of Vascular Nitric Oxide and Reactive Oxygen Species and Their Regulation (2019) · cites this paper as [837]
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Metadata
- Authors
- Schwarz, G.
- Venue
- Current Opinion in Chemical Biology
- Publisher
- Elsevier BV
- Published
- 2016-04
- Volume
- 31
- Pages
- 179-187
- ISSN
- 1367-5931 (ISSN-L)
- OpenAlex
- W2341653166
- Cited by
- 115
- References
- 56
- Citations by year
- 2026: 4 · 2025: 8 · 2024: 12 · 2023: 15 · 2022: 7 · 2021: 13 · 2020: 16 · 2019: 16 · 2018: 14 · 2017: 8 · 2016: 2
- FWCI
- 1.38
- Type
- journal article
- Language
- en
- Open access
- closed
- Locations
- Current Opinion in Chemical Biology (publishedVersion)
- PubMed (publishedVersion)
- Kölner Universitäts PublikationsServer (Universität zu Köln) (publishedVersion)
- Funders
- German Science Foundation
- Federal Ministry for Education and Research
- Center for Molecular Medicine Cologne
- Fonds der Chemischen Industrie
- Topics
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Clinical Biochemistry › Metabolism and Genetic Disorders
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Biochemistry › Metalloenzymes and iron-sulfur proteins
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Porphyrin Metabolism and Disorders
- Keywords
- molybdenum cofactor, molybdenum enzymes, sulfite oxidase deficiency, inborn errors of metabolism, molybdenum cofactor biosynthesis, pterin cofactor, nitrite reduction, nitric oxide, cysteine catabolism, disease mechanisms, treatment options
- MeSH terms
- Molybdenum Cofactors, Coenzymes, Cysteine, Cytosol, Disease, Humans, Metalloproteins, Mitochondria, Pteridines, Sulfites, Therapeutics
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