lysyl hydroxylase


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ly·syl hy·drox·y·lase

an enzyme that acts on specific lysyl residues in certain proteins (for example, collagens) with α-ketoglutarate and O2 to produce δ-hydroxylysyl residues, succinate, and CO2; this enzyme, which requires Fe2+ and ascorbate, is deficient in Ehlers-Danlos syndrome type VI.
References in periodicals archive ?
Mutations in the lysyl hydroxylase 1 gene that result in enzyme deficiency and the clinical phenotype of Ehlers-Danlos syndrome type VI.
In vivo inhibition of chick embryo lysyl hydroxylase by homogentisic acid: a proposed connective defect in alkaptonuria.
This study demonstrated that ascorbate improves the clinical status of patients with impaired collagen lysyl hydroxylase activity by enhancing lysyl and prolyl hydroxylation and total collagen production.