Cofactor-dependent maturation of mammalian sulfite oxidase links two mitochondrial import pathways
نویسندگان
چکیده
منابع مشابه
Cofactor-dependent maturation of mammalian sulfite oxidase links two mitochondrial import pathways.
Sulfite oxidase (SO) catalyses the metabolic detoxification of sulfite to sulfate within the intermembrane space of mitochondria. The enzyme follows a complex maturation pathway, including mitochondrial transport and processing, integration of two prosthetic groups, molybdenum cofactor (Moco) and heme, as well as homodimerisation. We have identified the sequential and cofactor-dependent maturat...
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A universal molybdenum-containing cofactor is necessary for the activity of all eukaryotic molybdoenzymes. In humans four such enzymes are known: Sulfite oxidase, xanthine oxidoreductase, aldehyde oxidase and a mitochondrial amidoxime reducing component. Of these, sulfite oxidase is the most important and clinically relevant one. Mutations in the genes MOCS1, MOCS2 or GPHN all encoding cofactor...
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While the existence of a molybdenum cofactor common to nitrate reductase, xanthine oxidase, and sulfite oxidase has been finnly established by studies from several laboratories (1-6), the nature of the molecule has not been elucidated. Information available to date has shown that the molybdenum cofactor complex is noncovalently attached to the proteins (2, 4) and is probably a small, dialyzable...
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Three mitochondria-encoded subunits form the catalytic core of cytochrome c oxidase, the terminal enzyme of the respiratory chain. COX1 and COX2 contain heme and copper redox centers, which are integrated during assembly of the enzyme. Defects in this process lead to an enzyme deficiency and manifest as mitochondrial disorders in humans. Here we demonstrate that COA6 is specifically required fo...
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ژورنال
عنوان ژورنال: Journal of Cell Science
سال: 2012
ISSN: 1477-9137,0021-9533
DOI: 10.1242/jcs.110114