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Pro-myostatin is secreted and retained in the ECM in an LTBP3-dependent manner and upon cleavage by furin, the mature growth factor is released from the complex with LTBP3.
LTBPs form multiple intra- and extra-cellular associations with divergent proteins. In addition, LTBPs play an important structural role in microfibril and elastic fibril assembly and organization. The backbone of the ECM is composed of fibrilar proteins, such as collagen, fibronectin, and elastin to which many adaptor proteins and proteoglycans associate, together forming a complex and dynamic 3-D structure.
The building blocks of elastic matrices are fibrillins and tropoelastin, but elastogenesis and maintenance of elastic fibers would be impossible without a number of other factors, including specific LTBPs and fibulins.