Definition: an enzyme that acts on specific lysyl residues in certain proteins (collagens) with a-ketoglutarate and O2 to produce ?-hydroxylysyl residues, succinate, and CO2; this enzyme, which requires Fe2+ and ascorbate, is deficient in Ehlers-Danlos syndrome type VI.
Synonym(s): lysyl 2-oxoglutarate dioxygenase
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