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Collagen synthesis, Col1A2 not mutated in Nancy normal synthesis of alpha 2 (I), triple helix, procollagen transported to Golgi Packaged into vesicles, glycosylated pro alpha chains disulfide bonds at C-terminal In Type I OI, no triple helix formed, triple helix, procollagen type of collagen Type I Collagen, mature collagen fibrils in staggered array crosslinking by lysyl oxidase collagen fibers, Pro alpha chain (don't associate because of telopeptides) prolyl hydroxylase lysyl hydroxylase proline and lysine residues hyrdoxylated, Type I Collagen formed from 2 alpha 1 (I) chains 1 alpha 2 (I) chains, In Type I OI, no triple helix formed caused by Mutation of glycine to amino acids with bulky side chain, In Type I OI, no triple helix formed leads to protein accumulation, Mutation of glycine to amino acids with bulky side chain leads to decreased secretion of collagen monomer