EC Number   |
General Information |
Reference |
|---|
    1.8.3.1 | malfunction |
defects in the enzyme cause a severe infant disease leading to early death with no efficient or costeffective therapy in sight |
741495 |
| 1.8.3.1 | malfunction |
enzyme absence confers reduced biomass accumulation in Arabidopsis plants exposed to carbon starvation. Enzyme impairment leads to a reduced sulfur reduction pathway under sucrose depletion and reduced biomass accumulation in plants grown on excess carbon supply |
764788 |
| 1.8.3.1 | malfunction |
enzyme knockdown animals move less resulting from a reduced peristalsis efficacy |
765457 |
| 1.8.3.1 | malfunction |
impairment in enzyme activity results in enhanced water consumption |
-, 765161 |
| 1.8.3.1 | malfunction |
in humans, sulfite oxidase deficiency is one of the most accepted causes of sulfite hypersensitivity and toxicity. A congenital deficiency of sulfite oxidase can cause an excessive accumulation of sulfite and lead to early death in infancy (usually between 2 and 6 years of age), or in neonatal cases, neurological abnormalities, mental retardation, intractable seizures, and ocular lens dislocation. Molybdenum cofactor deficiency, which compromises sulfite oxidase activity, results in profound mental retardation, brain damage, microcephaly, and spasticity. It has also been suggested that hypoxic-ischemic encephalopathy is due to molybdenum cofactor deficiency |
741809 |
| 1.8.3.1 | malfunction |
R309H and K322R mutations are responsible for isolated sulfite oxidase deficiency |
743226 |
| 1.8.3.1 | malfunction |
silencing of ZmSO could lead to seed germination delay upon sulfite exposure, but not under normal conditions |
743481 |
| 1.8.3.1 | malfunction |
the imbalanced sulfite level resulting from sulfite oxidase impairment confers a metabolic shift towards elevated reduced S-compounds, namely sulfide, S-amino acids (S-AA), Co-A and acetyl-CoA, followed by non-S-AA, nitrogen and carbon metabolite enhancement, including polar lipids. Exposing mutant plants to dark-induced carbon starvation result in a higher degradation of S-compounds, total AA, carbohydrates, polar lipids and total RNA in the mutant plants. Significantly, a failure to balance the carbon backbones is evident in the mutants, indicated by an increase in tricarboxylic acid cycle (TCA) cycle intermediates, whereas a decrease is shown in stressed wild-type plants. Sulfite oxidase deficiency is not necessarily lethal, unless other sulfite network enzymes are down-regulated or the capacity of the sulfite network enzymes in sulfite detoxification is exceeded. Sulfite oxidase mutation affects carbon metabolism in normal and dark-stressed plants. Extended dark stress leads to enhanced degradation of organic nitrogen, elevated ammonium and preference for lower C/N ratio metabolites in the sulfite oxidase mutants as compared with the wild-type plants. Phenotype, detailed overview |
743565 |
| 1.8.3.1 | metabolism |
the enzyme is involved in the sulfite pathway in plants, overview |
743424 |
| 1.8.3.1 | more |
the catalytic site of SO consists of a molybdenum ion bound to the dithiolene sulfurs of one molybdopterin (MPT) molecule, carrying two oxygen ligands, and is further coordinated by the thiol sulfur of a conserved cysteine residue |
741618 |
