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25 In line with the role of ADAMTS-2 as an important procollagen type I N-endopeptidase, 9,12,17 we found abnormal processing of the N-propeptide of type I procollagen, with accumulation of pNα1(I) and pNα2(I) procollagen chains and near-absence of pCα1(I) and pCα2(I) procollagen chains in patients’ derived fibroblasts

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While our understanding of the regulation of the enzymes of the pathway has greatly increased, some aspects such as the moonlighting functions remain to be fully elucidated