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Gene Review

SUOX  -  sulfite oxidase

Homo sapiens

Synonyms: Sulfite oxidase, mitochondrial
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Disease relevance of SUOX


Psychiatry related information on SUOX

  • Isolated sulfite oxidase deficiency is a rare autosomal recessive disease, characterized by severe neurological abnormalities, seizures, mental retardation, and dislocation of the ocular lenses, that often leads to death in infancy [5].

High impact information on SUOX

  • The premier discoveries to emanate from my laboratory have been the sulfite oxidase, the several superoxide dismutases, the manganese catalase, and the catalase/peroxidase [6].
  • Sulfite oxidase activity in skin fibroblasts average 1.07 nmol of cytochrome d reduced per milligram of protein per minute in control lines; it was not detectable (less than 5 per cent) in the patient [7].
  • Molybdenum cofactor deficiency is a devastating disease with affected patients displaying the symptoms of a combined deficiency of sulfite oxidase and xanthine dehydrogenase [8].
  • Use of conditioned media indicated that a relatively stable, diffusible precursor produced by group B cells could be used to repair sulfite oxidase in group A recipient cells [8].
  • The ability of the patient's liver to synthesize the specific molybdenum cofactor required for activation of de-molybdo sulfite oxidase also appears to have been unimpaired [9].

Chemical compound and disease context of SUOX


Biological context of SUOX


Anatomical context of SUOX

  • The deficiencies can be diagnosed prenatally by monitoring sulfite oxidase activity in chorionic villus sampling (CVS) tissue [1].
  • This precedes severe cystic encephalomalacia and suggests that the energy failure associated with neuronal dysfunction and myelin disintegration occurs early in isolated sulfite oxidase deficiency [18].
  • Effect of sulfite on red blood cell deformability ex vivo and in normal and sulfite oxidase-deficient rats in vivo [19].
  • Expressions of N-methyl-D: -aspartate receptors NR2A and NR2B subunit proteins in normal and sulfite-oxidase deficient rat's hippocampus: effect of exogenous sulfite ingestion [20].
  • Liver tissue and hepatocytes, however, exhibit high activities of sulfite oxidase [21].

Associations of SUOX with chemical compounds


Other interactions of SUOX


Analytical, diagnostic and therapeutic context of SUOX


  1. Prenatal diagnosis of molybdenum cofactor deficiency and isolated sulfite oxidase deficiency. Johnson, J.L. Prenat. Diagn. (2003) [Pubmed]
  2. In vitro incorporation of nascent molybdenum cofactor into human sulfite oxidase. Leimkühler, S., Rajagopalan, K.V. J. Biol. Chem. (2001) [Pubmed]
  3. Isolated sulfite oxidase deficiency: a case report with a novel mutation and review of the literature. Tan, W.H., Eichler, F.S., Hoda, S., Lee, M.S., Baris, H., Hanley, C.A., Grant, P.E., Krishnamoorthy, K.S., Shih, V.E. Pediatrics (2005) [Pubmed]
  4. Sulfite oxidase deficiency: clinical, neuroradiologic, and biochemical features in two new patients. Brown, G.K., Scholem, R.D., Croll, H.B., Wraith, J.E., McGill, J.J. Neurology (1989) [Pubmed]
  5. DNA-based diagnosis of isolated sulfite oxidase deficiency by denaturing high-performance liquid chromatography. Lam, C.W., Li, C.K., Lai, C.K., Tong, S.F., Chan, K.Y., Ng, G.S., Yuen, Y.P., Cheng, A.W., Chan, Y.W. Mol. Genet. Metab. (2002) [Pubmed]
  6. With the help of giants. Fridovich, I. Annu. Rev. Biochem. (2003) [Pubmed]
  7. Sulfite oxidase deficiency. Biochemical and clinical investigations of a hereditary metabolic disorder in sulfur metabolism. Shih, V.E., Abroms, I.F., Johnson, J.L., Carney, M., Mandell, R., Robb, R.M., Cloherty, J.P., Rajagopalan, K.V. N. Engl. J. Med. (1977) [Pubmed]
  8. Molybdenum cofactor biosynthesis in humans. Identification of two complementation groups of cofactor-deficient patients and preliminary characterization of a diffusible molybdopterin precursor. Johnson, J.L., Wuebbens, M.M., Mandell, R., Shih, V.E. J. Clin. Invest. (1989) [Pubmed]
  9. Human sulfite oxidase deficiency. Characterization of the molecular defect in a multicomponent system. Johnson, J.L., Rajagopalan, K.V. J. Clin. Invest. (1976) [Pubmed]
  10. Escherichia coli MoeA and MogA. Function in metal incorporation step of molybdenum cofactor biosynthesis. Nichols, J., Rajagopalan, K.V. J. Biol. Chem. (2002) [Pubmed]
  11. Sulfite oxidase activity in Thiobacillus novellus. Southerland, W.M., Toghrol, F. J. Bacteriol. (1983) [Pubmed]
  12. Visual evoked potentials in normal and sulfite oxidase deficient rats exposed to ingested sulfite. Küçükatay, V., Hacioğlu, G., Savcioğlu, F., Yargiçoğlu, P., Ağar, A. Neurotoxicology (2006) [Pubmed]
  13. Health effects of atmospheric sulfur dioxide and dietary sulfites. The fallacy of typology. Hickey, R.J., Clelland, R.C., Bowers, E.J., Boyce, D.E. Arch. Environ. Health (1976) [Pubmed]
  14. A novel mutation in neonatal isolated sulphite oxidase deficiency. Lee, H.F., Mak, B.S., Chi, C.S., Tsai, C.R., Chen, C.H., Shu, S.G. Neuropediatrics. (2002) [Pubmed]
  15. The 1.2 A structure of the human sulfite oxidase cytochrome b(5) domain. Rudolph, M.J., Johnson, J.L., Rajagopalan, K.V., Kisker, C. Acta Crystallogr. D Biol. Crystallogr. (2003) [Pubmed]
  16. Mutations in the molybdenum cofactor biosynthetic genes MOCS1, MOCS2, and GEPH. Reiss, J., Johnson, J.L. Hum. Mutat. (2003) [Pubmed]
  17. Human sulfite oxidase R160Q: identification of the mutation in a sulfite oxidase-deficient patient and expression and characterization of the mutant enzyme. Garrett, R.M., Johnson, J.L., Graf, T.N., Feigenbaum, A., Rajagopalan, K.V. Proc. Natl. Acad. Sci. U.S.A. (1998) [Pubmed]
  18. Proton Magnetic Resonance Spectroscopy And Diffusion-weighted Imaging In Isolated Sulfite Oxidase Deficiency. Eichler, F., Tan, W.H., Shih, V.E., Grant, P.E., Krishnamoorthy, K. J. Child Neurol. (2006) [Pubmed]
  19. Effect of sulfite on red blood cell deformability ex vivo and in normal and sulfite oxidase-deficient rats in vivo. Bor-Kucukatay, M., Kucukatay, V., Agar, A., Baskurt, O.K. Arch. Toxicol. (2005) [Pubmed]
  20. Expressions of N-methyl-D: -aspartate receptors NR2A and NR2B subunit proteins in normal and sulfite-oxidase deficient rat's hippocampus: effect of exogenous sulfite ingestion. Oztürk, O.H., Küçükatay, V., Yönden, Z., Ağar, A., Bağci, H., Delibaş, N. Arch. Toxicol. (2006) [Pubmed]
  21. Effect of sulfite on the energy metabolism of mammalian tissues in correlation to sulfite oxidase activity. Beck-Speier, I., Hinze, H., Holzer, H. Biochim. Biophys. Acta (1985) [Pubmed]
  22. The role of tyrosine 343 in substrate binding and catalysis by human sulfite oxidase. Wilson, H.L., Rajagopalan, K.V. J. Biol. Chem. (2004) [Pubmed]
  23. The sequence of squash NADH:nitrate reductase and its relationship to the sequences of other flavoprotein oxidoreductases. A family of flavoprotein pyridine nucleotide cytochrome reductases. Hyde, G.E., Crawford, N.M., Campbell, W.H. J. Biol. Chem. (1991) [Pubmed]
  24. Distribution and pathophysiologic role of molybdenum-containing enzymes. Moriwaki, Y., Yamamoto, T., Higashino, K. Histol. Histopathol. (1997) [Pubmed]
  25. Mitochondrial antigen/antibody systems in primary biliary cirrhosis: revisited. Berg, P.A., Klein, R. Liver (1995) [Pubmed]
  26. A review of the clinical presentation and laboratory findings in two uncommon hereditary disorders of sulfur amino acid metabolism, beta-mercaptolactate cysteine disulfideuria and sulfite oxidase deficiency. Crawhall, J.C. Clin. Biochem. (1985) [Pubmed]
  27. Molecular cloning of human liver sulfite oxidase. Garrett, R.M., Bellissimo, D.B., Rajagopalan, K.V. Biochim. Biophys. Acta (1995) [Pubmed]
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