LysylHydroxylasen
Lysyl hydroxylases are a group of iron(II)- and 2-oxoglutarate–dependent dioxygenases that catalyze the post-translational hydroxylation of lysine residues in collagen and related extracellular matrix proteins. The resulting hydroxylysine residues are important sites for subsequent glycosylation and for the formation of mature collagen cross-links, influencing the stability and mechanical properties of connective tissue.
In humans, three isoforms are described: lysyl hydroxylase 1 (PLOD1), lysyl hydroxylase 2 (PLOD2), and lysyl hydroxylase
The enzymatic reaction involves hydroxylation of specific lysine residues within procollagen chains. Hydroxylysine residues serve as
Genetic and clinical relevance is seen in several inherited connective tissue disorders. PLOD1 mutations cause kyphoscoliosis-type