182 Cross - Linking Defect in Aneurysm - Prone Mice

Mice wi th alleles a t the X-chromosomal locus, Mottled, have connective tissue abnormali t ies tha t are, in m a n y respects, similar to those found in la thyr i t ic animals. Like animals raised on lathyrogens, these mice have aort ic aneurysms, reduced tensile s t rength of the skin, and bone abnormali t ies (1). D a t a is presented to indicate tha t the low tensile s t rength is due to impaired format ion of cross-links in collagen and elastin, and tha t cross-linking is defect ive because of failure to generate lysine-derived aldehydes (allysine) required for cross-link formation.