ADAMTS-like protein 3 (ADAMTSL3)

ADAMTS-like protein 3 (ADAMTSL3) is a secreted, multidomain glycoprotein of the ADAMTS-like family, localized primarily in the extracellular matrix (ECM)[2][3]. Unlike the ADAMTS proteases, ADAMTSL3 lacks enzymatic activity but retains a similar domain structure with thrombospondin type I repeats, which facilitate matrix and microfibril interactions[1][2]. It is involved in regulating microfibril assembly by binding fibrillin-1 and modulating TGFβ activity in the ECM, thereby influencing ECM remodeling, fibrosis, and cellular differentiation, particularly in the heart and vasculature[1][3]. ADAMTSL3 is highly expressed in heart, skeletal muscle, liver, kidney, and some connective tissues, and has been implicated as a genetic contributor to normal growth (e.g., height), certain connective tissue disorders, cardiovascular pathologies, and psychiatric diseases. Animal and human studies suggest a cardioprotective function mediated by limitation of TGFβ signaling and regulation of fibroblast activation, ECM deposition, and collagen production. Emerging evidence supports its potential as both a disease biomarker and a therapeutic target in cardiovascular medicine, although no drugs currently target ADAMTSL3 directly[1][3][2][4].

Other names

ADAMTSL3ADAMTS-like protein 3KIAA1233ADAMTSL-3punctin-2Punctin-2a disintegrin-like and metalloprotease domain with thrombospondin type I motifs-like 3