ADAMTS, cysteine-rich domain 2
IPR041645
Definition
This cysteine rich domain is found in a variety of ADAMTS peptidases. Proteolysis of the extracellular matrix plays a critical role in establishing tissue architecture during development and in tissue degradation in diseases such as cancer, arthritis, Alzheimer's disease and a variety of inflammatory conditions [[cite:PMID:9390552], [cite:PMID:34268335]]. The proteolytic enzymes responsible for this process are members of diverse protease families, including the secreted zinc metalloproteases (MPs) PMID:9390552. ADAM-TS (A Disintegrin and Metalloproteinase with Thrombospondin Motifs) is closely related to the ADAM family (A Disintegrin and Metalloproteinase) and is a subfamily of the MP family, consists of at least 20 members sharing a high degree of sequence similarity and conserved domain organisation [[cite:PMID:8995297], [cite:PMID:10464288]]. The defining domains of the ADAM-TS family are (from N- to C-termini) a pre-pro metalloprotease domain of the reprolysin type, a snake venom disintegrin-like domain, a thrombospondin type-I (TS) module, a cysteine-rich region, and a cysteine-free (spacer) domain PMID:10464288. Domain organisation following the spacer domain C terminus shows some variability in certain ADAM-TS members, principally in the number of additional TS domains. These enzymes have a wide-spectrum role in vascular biology and cardiovascular pathophysiology PMID:30905657. Members of the ADAM-TS family have been implicated in a range of diseases [[cite:PMID:17265492], [cite:PMID:29885460], [cite:PMID:34268335]]. For instance, members of this family have been found to participate directly in processes in the central nervous system (CNS) such as the regulation of brain plasticity PMID:32150898. ADAM-TS1 is reported to be involved in inflammation and cancer cachexia PMID:8995297, whilst recessively inherited ADAM-TS2 mutations cause Ehlers-Danlos syndrome type VIIC, a disorder characterised clinically by severe skin fragility PMID:10417273. ADAM-TS4 is an aggrecanase involved in arthritic destruction of cartilage PMID:10356395.
19 human proteins with this domain
Each is a reviewed human protein in the Seroatlas serome and a potential autoantibody target; this domain groups them into one antibody-relevant category. Every entry links to its own record.
Loading the interactive Seroatlas explorer...