Lysyl hydroxylase

Figure 28-11. The prolyl hydroxylase reaction. The substrate is a proline-rich peptide. During the course of the reaction, molecular oxygen is incorporated into both succinate and proline. Lysyl hydroxylase catalyzes an analogous reaction.

Lysyl hydroxylase Ehlers-Danlos syndrome type VI (MIM 225400)... 

Myllyla, R., Schubotz, L.M., Weser, U. and Kivirikko, K.I. (1979). Involvement of superoxide in the prolyl and lysyl hydroxylase reactions. Biochem. Biophy. Res. Commun. 89, 98-102. 

The a-ketoacid-dependent enzymes are distinguished from other non-haem iron enzymes by their absolute requirement for an a-ketoacid cofactor as well as Fe(II) and O2 for activity. They catalyse two types of reaction (Table 2.3), hydroxyla-tion and oxidation. In both, the a-ketoglutarate is decarboxylated and one oxygen atom introduced into the succinate formed in the hydroxylases, the other oxygen atom is introduced into the substrate, while in the oxidases it is found in water, together with the cyclized product. In general these enzymes require one equivalent of Fe(II) an a-ketoacid, usually a-ketoglutarate and ascorbate. Examples of these enzymes include proline 4-hydroxylase, prolyl and lysyl hydroxylase, which... 

Figure 10 Schematic illustration of the posttranslational enzymes and related proteins. P4H a subunits have three isoforms. Each has three domains. The substrate-binding domain is in the middle. The catalytic domain is at the C-terminal end. Lysyl hydroxylase-3 (LH3) has two different catalytic activities. The N-terminal domain has the glucosyltransferase activity and the C-terminal domain has the hydroxylase activity. LH1 and LH2 also have similar domain structures but the glucosyltransferase activities are not detected in vitro.

Selected prolines and lysines are hydroxylated by prolyl and lysyl hydroxylases. These enzymes, located in the RER, require ascorbate (vitamin C), deficiency of which produces scurvy. 

GLUTAMATE DEHYDROGENASE GLUTAMATE SYNTHASE a-KETOGLUTARATE DEHYDROGENASE LEUCINE AMINOTRANSFERASE LYSINE 6-AMINOTRANSFERASE LYSYL HYDROXYLASE... 

KINETIC ISOTOPE EFFECT LYSYL ENDOPEPTIDASE LYSYL HYDROXYLASE LYSYL OXIDASE Lysyl residue,... 

LYSINE MONOOXYGENASE LYSYL HYDROXYLASE MANDELATE 4-MONOOXYGENASE MELILOTATE HYDROXYLASE METHANE MONOOXYGENASE... 

II, III, IV, and VII collagens. Other defects involve lysyl hydroxylase and procollagen N-proteinase.a-d... 

Saika S, Ooshima A, Hashizume N, Yamanaka O, Tanaka S, Okada Y, Kobata S. Effect of lysyl hydroxylase inhibitor, minoxidil, on ultrastructure and behavior of cultured rabbit subconjunctival fibroblasts. Graefe s Archive for Clinical Experimental Ophthalmology 1995, 233, 347-353. 

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