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1.8.5.8: eukaryotic sulfide quinone oxidoreductase

This is an abbreviated version!
For detailed information about eukaryotic sulfide quinone oxidoreductase, go to the full flat file.

Reaction

hydrogen sulfide
+
glutathione
+
a quinone
=
S-sulfanylglutathione
+
a quinol

Synonyms

glutathione:CoQ reductase, HMT2, ScSQR, SQOR, SQR, Sqrdl, sulfide : quinone oxidoreductase, sulfide quinone oxidoreductase, sulfide:quinone oxidoreductase

ECTree

     1 Oxidoreductases
         1.8 Acting on a sulfur group of donors
             1.8.5 With a quinone or similar compound as acceptor
                1.8.5.8 eukaryotic sulfide quinone oxidoreductase

Engineering

Engineering on EC 1.8.5.8 - eukaryotic sulfide quinone oxidoreductase

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PROTEIN VARIANTS
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
E213K
naturally occuring mutation the mutation affects a residue that is remote from the active site, but predicted to disrupt hydrogen bonding with neighboring arginine residues. The mutation leads to greatly diminished SQOR levels in tissues expressing the E213K mutation
additional information
a patient is homozygous for a single base pair deletion (c446delT) in the SQOR gene, predicted to lead to mRNA degradation or production of nonfunctional enzyme due to the resulting frameshift, SQOR is not detected in fibroblasts carrying the deletion mutation