Nozickova 2017 MiPschool Obergurgl

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Nozickova K
Sulfite oxidase.

Link: MitoEAGLE

Nozickova K, Sobotka O (2017)

Event: MiPschool Obergurgl 2017

COST Action MitoEAGLE

Sulfite oxidase (SO) is a dimeric enzyme, located in the intermembrane space of mitochondria, with each monomer containing a single Mo cofactor and cyt b5-type heme [1]. SO catalyzes the oxidation of sulfite to sulfate as the terminal step in the metabolism of sulfur amino acids and is vital for human health. Inherited mutations in SO result in severe neurological problems, stunted brain growth, and early death [2].

Function: SO catalyzes the terminal reaction in the oxidative degradation of SULFUR AMINO ACIDS with the formation of a sulfate, electrons pass to cytochrom c and are further utilized in respiratory chain.

Sulfite + O2 + H2O --> Sulfate + H2O2

Localization: The level of expression of SO differs in various tissues with main predominant localization in liver, kidney, skeletal muscle, heart, placenta, and brain in humans and liver, kidney, heart, brain, and lung in rats [3].

Deficiency: SO is vital for metabolic pathways of sulfur amino acids (cysteine and methionine). Complete lack of this enzyme, typically caused by gene mutation, leads to lethal disease called sulfite oxidase deficiency characterized by neurological abnormalities with brain atrophy.


Bioblast editor: Kandolf G O2k-Network Lab: CZ Hradec Kralove Cervinkova Z


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Affiliations

Dept Physiol, Fac Medicine Hradec Kralove, Charles Univ Prague, Czech Republic.- nozickok@lfhk.cuni.cz

References

  1. Chaudhury PK, Das SK, Sarkar S (1996) Inhibition patterns of a model complex mimicking the reductive half-reaction of sulphite oxidase. Biochem J 319:953-9.
  2. Seidahmed MZ, Alyamani EA, Rashed MS, Saadallah AA, Abdelbasit OB, Shaheed MM, Rasheed A, Hamid FA, Sabry MA (2005) Total truncation of the molybdopterin/dimerization domains of SUOX protein in an Arab family with isolated sulfite oxidase deficiency. Am J Med Genet A 136:5-9.
  3. Woo WH, Yang H, Wong KP, Halliwell B (2003) Sulphite oxidase gene expression in human brain and in other human and rat tissues. Biochem Biophys Res Commun 305:619-23.