The under-hydroxylation of lysines at triple-helical domain cross-linking sites, and resulting inversed ratio of HP/LP cross-links in bone collagen closely resembles the collagen pathology in Ehlers-Danlos Syndrome type VI (EDS VI) caused by PLOD1 (encodes LH1) mutations (for a review see [36]). Here, PLOD1 is linked to kyphoscoliotic Ehlers-Danlos syndrome.