Dysregulation of action of TGFβ and related molecules, such as BMPs (BMP2, BMP4, and BMP6) and CTGF (a mediator of BMP activity) in damaged tendons has been well documented in human and animal tendinopathies where the excessive presence of BMPs can lead to increased synthesis and deposition of proteoglycans in the tendon [29, 34]. Here, CLN5 is linked to disease of the tendon.