1. Wuebbens MM, Liu MT, Rajagopalan K, Schindelin H. Insights into molybdenum cofactor deficiency provided by the crystal structure of the molybdenum cofactor biosynthesis protein MoaC. Structure Fold Des. 2000;8(7):709-718. (PubMed)
2. Food and Nutrition Board, Institute of Medicine. Molybdenum. In: Dietary reference intakes for vitamin A, vitamin K, boron, chromium, copper, iodine, iron, manganese, molybdenum, nickel, silicon, vanadium, and zinc. Washington, D.C.: National Academy Press; 2001:420-441. (National Academy Press)
3. Schwarz G, Mendel RR, Ribbe MW. Molybdenum cofactors, enzymes and pathways. Nature. 2009;460(7257):839-847. (PubMed)
4. Wang J, Krizowski S, Fischer-Schrader K, et al. Sulfite oxidase catalyzes single-electron transfer at molybdenum domain to reduce nitrite to nitric oxide. Antioxid Redox Signal. 2015;23(4):283-294. (PubMed)
5. Eckhert C. Other trace elements In: Shils ME, Shike M, Ross AC, Caballero B, Cousins RJ, eds. Modern Nutrition in Health and Disease. 10th ed. Philadelphia: Lippincott, Williams & Wilkins; 2006:338-350.
6. Wahl B, Reichmann D, Niks D, et al. Biochemical and spectroscopic characterization of the human mitochondrial amidoxime reducing components hmARC-1 and hmARC-2 suggests the existence of a new molybdenum enzyme family in eukaryotes. J Biol Chem. 2010;285(48):37847-37859. (PubMed)
7. Havemeyer A, Bittner F, Wollers S, Mendel R, Kunze T, Clement B. Identification of the missing component in the mitochondrial benzamidoxime prodrug-converting system as a novel molybdenum enzyme. J Biol Chem. 2006;281(46):34796-34802. (PubMed)
8. Plitzko B, Ott G, Reichmann D, et al. The involvement of mitochondrial amidoxime reducing components 1 and 2 and mitochondrial cytochrome b5 in N-reductive metabolism in human cells. J Biol Chem. 2013;288(28):20228-20237. (PubMed)
9. Ott G, Havemeyer A, Clement B. The mammalian molybdenum enzymes of mARC. J Biol Inorg Chem. 2015;20(2):265-275. (PubMed)
10. Sparacino-Watkins CE, Tejero J, Sun B, et al. Nitrite reductase and nitric-oxide synthase activity of the mitochondrial molybdopterin enzymes mARC1 and mARC2. J Biol Chem. 2014;289(15):10345-10358. (PubMed)
11. Mayr SJ, Mendel RR, Schwarz G. Molybdenum cofactor biology, evolution and deficiency. Biochim Biophys Acta Mol Cell Res. 2021;1868(1):118883. (PubMed)
12. Beedham C. Molybdenum hydroxylases as drug-metabolizing enzymes. Drug Metab Rev. 1985;16(1-2):119-156. (PubMed)
13. Zannolli R, Micheli V, Mazzei MA, et al. Hereditary xanthinuria type II associated with mental delay, autism, cortical renal cysts, nephrocalcinosis, osteopenia, and hair and teeth defects. J Med Genet. 2003;40(11):e121. (PubMed)
14. Fujiwara Y, Kawakami Y, Shinohara Y, Ichida K. A case of hereditary xanthinuria type 1 accompanied by bilateral renal calculi. Intern Med. 2012;51(14):1879-1884. (PubMed)
15. Turnlund JR, Keyes WR. Dietary molybdenum: Effect on copper absorption, excretion, and status in young men. In: Roussel AM, ed. Trace Elements in Man and Animals. Vol 10. New York: Kluwer Academic Press.; 2000:951-953.
16. Suttle NF. Copper imbalances in ruminants and humans: unexpected common ground. Adv Nutr. 2012;3(5):666-674. (PubMed)
17. Lopez-Alonso M, Miranda M. Copper supplementation, a challenge in cattle. Animals (Basel). 2020;10(10):1890. (PubMed)
18. Helz GR, Erickson BE. Extraordinary stability of copper(I)-tetrathiomolybdate complexes: possible implications for aquatic ecosystems. Environ Toxicol Chem. 2011;30(1):97-102. (PubMed)
19. Alvarez HM, Xue Y, Robinson CD, et al. Tetrathiomolybdate inhibits copper trafficking proteins through metal cluster formation. Science. 2010;327(5963):331-334. (PubMed)
20. Brewer GJ, Askari F, Dick RB, et al. Treatment of Wilson's disease with tetrathiomolybdate: V. Control of free copper by tetrathiomolybdate and a comparison with trientine. Transl Res. 2009;154(2):70-77. (PubMed)
21. Redman BG, Esper P, Pan Q, et al. Phase II trial of tetrathiomolybdate in patients with advanced kidney cancer. Clin Cancer Res. 2003;9(5):1666-1672. (PubMed)
22. Gartner EM, Griffith KA, Pan Q, et al. A pilot trial of the anti-angiogenic copper lowering agent tetrathiomolybdate in combination with irinotecan, 5-flurouracil, and leucovorin for metastatic colorectal cancer. Invest New Drugs. 2009;27(2):159-165. (PubMed)
23. Jain S, Cohen J, Ward MM, et al. Tetrathiomolybdate-associated copper depletion decreases circulating endothelial progenitor cells in women with breast cancer at high risk of relapse. Ann Oncol. 2013;24(6):1491-1498. (PubMed)
24. Hou G, Abrams GD, Dick R, Brewer GJ. Efficacy of tetrathiomolybdate in a mouse model of multiple sclerosis. Transl Res. 2008;152(5):239-244. (PubMed)
25. Wei H, Zhang WJ, McMillen TS, Leboeuf RC, Frei B. Copper chelation by tetrathiomolybdate inhibits vascular inflammation and atherosclerotic lesion development in apolipoprotein E-deficient mice. Atherosclerosis. 2012;223(2):306-313. (PubMed)
26. Askari F, Innis D, Dick RB, et al. Treatment of primary biliary cirrhosis with tetrathiomolybdate: results of a double-blind trial. Transl Res. 2010;155(3):123-130. (PubMed)
27. Abumrad NN, Schneider AJ, Steel D, Rogers LS. Amino acid intolerance during prolonged total parenteral nutrition reversed by molybdate therapy. Am J Clin Nutr. 1981;34(11):2551-2559. (PubMed)
28. Reiss J, Hahnewald R. Molybdenum cofactor deficiency: Mutations in GPHN, MOCS1, and MOCS2. Hum Mutat. 2011;32(1):10-18. (PubMed)
29. Tan WH, Eichler FS, Hoda S, et al. Isolated sulfite oxidase deficiency: a case report with a novel mutation and review of the literature. Pediatrics. 2005;116(3):757-766. (PubMed)
30. Shalata A, Mandel H, Dorche C, et al. Prenatal diagnosis and carrier detection for molybdenum cofactor deficiency type A in northern Israel using polymorphic DNA markers. Prenat Diagn. 2000;20(1):7-11. (PubMed)
31. Kikuchi K, Hamano S, Mochizuki H, Ichida K, Ida H. Molybdenum cofactor deficiency mimics cerebral palsy: differentiating factors for diagnosis. Pediatr Neurol. 2012;47(2):147-149. (PubMed)
32. Atwal PS, Scaglia F. Molybdenum cofactor deficiency. Mol Genet Metab. 2016;117(1):1-4. (PubMed)
33. Johnson JL. Prenatal diagnosis of molybdenum cofactor deficiency and isolated sulfite oxidase deficiency. Prenat Diagn. 2003;23(1):6-8. (PubMed)
34. Hughes EF, Fairbanks L, Simmonds HA, Robinson RO. Molybdenum cofactor deficiency-phenotypic variability in a family with a late-onset variant. Dev Med Child Neurol. 1998;40(1):57-61. (PubMed)
35. Vijayakumar K, Gunny R, Grunewald S, et al. Clinical neuroimaging features and outcome in molybdenum cofactor deficiency. Pediatr Neurol. 2011;45(4):246-252. (PubMed)
36. Alkufri F, Harrower T, Rahman Y, et al. Molybdenum cofactor deficiency presenting with a parkinsonism-dystonia syndrome. Mov Disord. 2013;28(3):399-401. (PubMed)
37. Scelsa B, Gasperini S, Righini A, Iascone M, Brazzoduro VG, Veggiotti P. Mild phenotype in Molybdenum cofactor deficiency: A new patient and review of the literature. Mol Genet Genomic Med. 2019;7(6):e657. (PubMed)
38. Mills PB, Footitt EJ, Ceyhan S, et al. Urinary AASA excretion is elevated in patients with molybdenum cofactor deficiency and isolated sulphite oxidase deficiency. J Inherit Metab Dis. 2012;35(6):1031-1036. (PubMed)
39. Footitt EJ, Heales SJ, Mills PB, Allen GF, Oppenheim M, Clayton PT. Pyridoxal 5'-phosphate in cerebrospinal fluid; factors affecting concentration. J Inherit Metab Dis. 2011;34(2):529-538. (PubMed)
40. Struys EA, Nota B, Bakkali A, Al Shahwan S, Salomons GS, Tabarki B. Pyridoxine-dependent epilepsy with elevated urinary α-amino adipic semialdehyde in molybdenum cofactor deficiency. Pediatrics. 2012;130(6):e1716-1719. (PubMed)
41. Johnson JL, Duran M. Molybdenum cofactor deficiency and isolated sulfite deficiency. In: Scriver RC, ed. Metabolic and molecular bases of inherited disease. New York: Mcgraw-Hill; 2001:3163-3177.
42. Schwahn BC, Van Spronsen FJ, Belaidi AA, et al. Efficacy and safety of cyclic pyranopterin monophosphate substitution in severe molybdenum cofactor deficiency type A: a prospective cohort study. Lancet. 2015;386(10007):1955-1963. (PubMed)
43. Veldman A, Santamaria-Araujo JA, Sollazzo S, et al. Successful treatment of molybdenum cofactor deficiency type A with cPMP. Pediatrics. 2010;125(5):e1249-1254. (PubMed)
44. Hitzert MM, Bos AF, Bergman KA, et al. Favorable outcome in a newborn with molybdenum cofactor type A deficiency treated with cPMP. Pediatrics. 2012;130(4):e1005-1010. (PubMed)
45. US Food and Drug Administration. FDA Approves First Treatment for Molybdenum Cofactor Deficiency Type A. February 26, 2021. Available at: https://www.fda.gov/news-events/press-announcements/fda-approves-first-treatment-molybdenum-cofactor-deficiency-type. Accessed 6/25/21.
46. Jakubiczka-Smorag J, Santamaria-Araujo JA, Metz I, et al. Mouse model for molybdenum cofactor deficiency type B recapitulates the phenotype observed in molybdenum cofactor deficient patients. Hum Genet. 2016;135(7):813-826. (PubMed)
47. Turnlund JR, Keyes WR, Peiffer GL. Molybdenum absorption, excretion, and retention studied with stable isotopes in young men at five intakes of dietary molybdenum. Am J Clin Nutr. 1995;62(4):790-796. (PubMed)
48. Turnlund JR, Keyes WR, Peiffer GL, Chiang G. Molybdenum absorption, excretion, and retention studied with stable isotopes in young men during depletion and repletion. Am J Clin Nutr. 1995;61(5):1102-1109. (PubMed)
49. US Food and Drug Administration. Daily Value and Percent Daily Value: Changes on the New Nutrition and Supplement Facts Labels. March 2020. Available at: https://www.fda.gov/food/new-nutrition-facts-label/daily-value-new-nutrition-and-supplement-facts-labels#referenceguide. Accessed 5/28/21.
50. Nouri M, Chalian H, Bahman A, et al. Nail molybdenum and zinc contents in populations with low and moderate incidence of esophageal cancer. Arch Iran Med. 2008;11(4):392-396. (PubMed)
51. Ray SS, Das D, Ghosh T, Ghosh AK. The levels of zinc and molybdenum in hair and food grain in areas of high and low incidence of esophageal cancer: a comparative study. Glob J Health Sci. 2012;4(4):168-175. (PubMed)
52. Blot WJ, Li JY, Taylor PR, et al. Nutrition intervention trials in Linxian, China: supplementation with specific vitamin/mineral combinations, cancer incidence, and disease-specific mortality in the general population. J Natl Cancer Inst. 1993;85(18):1483-1492. (PubMed)
53. Qiao YL, Dawsey SM, Kamangar F, et al. Total and cancer mortality after supplementation with vitamins and minerals: follow-up of the Linxian General Population Nutrition Intervention Trial. J Natl Cancer Inst. 2009;101(7):507-518. (PubMed)
54. Wang SM, Taylor PR, Fan JH, et al. Effects of nutrition intervention on total and cancer mortality: 25-year post-trial follow-up of the 5.25-year Linxian Nutrition Intervention Trial. J Natl Cancer Inst. 2018;110(11):1229-1238. (PubMed)
55. Huang B, Zhao Y, Sun W, et al. Relationships between distributions of longevous population and trace elements in the agricultural ecosystem of Rugao County, Jiangsu, China. Environ Geochem Health. 2009;31(3):379-390. (PubMed)
56. Lv J, Wang W, Krafft T, Li Y, Zhang F, Yuan F. Effects of several environmental factors on longevity and health of the human population of Zhongxiang, Hubei, China. Biol Trace Elem Res. 2011;143(2):702-716. (PubMed)
57. Mills CF, Davis GK. Molybdenum. In: Mertz W, ed. Trace elements in human and animal nutrition. 5th ed. San Diego: Academic Press; 1987:429-463.
58. National Institutes of Health. Dietary Supplement Label Database. Version 7.0.12. August 2020. Available at: https://dsld.od.nih.gov/dsld. Accessed 5/28/21.
59. Hardy G, Menendez AM, Manzanares W. Trace element supplementation in parenteral nutrition: pharmacy, posology, and monitoring guidance. Nutrition. 2009;25(11-12):1073-1084. (PubMed)
60. Stehle P, Stoffel-Wagner B, Kuhn KS. Parenteral trace element provision: recent clinical research and practical conclusions. Eur J Clin Nutr. 2016;70(8):886-893. (PubMed)
61. Walravens PA, Moure-Eraso R, Solomons, CC, Chapell, R, Bentley G. Biochemical abnormalities in workers exposed to molybdenum dust. Arch Environ Health. 1979;34(5):302-308. (PubMed)
62. Vyskocil A, Viau C. Assessment of molybdenum toxicity in humans. J Appl Toxicol. 1999;19(3):185-192. (PubMed)
63. Momcilovic B. A case report of acute human molybdenum toxicity from a dietary molybdenum supplement--a new member of the "Lucor metallicum" family. Arh Hig Rada Toksikol. 1999;50(3):289-297. (PubMed)
64. Boles JW, Klaassen CD. Effects of molybdate and pentachlorophenol on the sulfation of acetaminophen. Toxicology. 2000;146(1):23-35. (PubMed)