Secreted. Associated with the extracellular matrix
Clonality
polyclonal
Description
This gene encodes a member of the ADAMTS (a disintegrin and metalloproteinase with thrombospondin motifs) protein family. Members of the family share several distinct protein modules, including a propeptide region, a metalloproteinase domain, a disintegrin-like domain, and a thrombospondin type 1 (TS) motif. Individual members of this family differ in the number of C-terminal TS motifs, and some have unique C-terminal domains. The enzyme encoded by this gene excises the N-propeptide of type I, type II and type V procollagens. Mutations in this gene cause Ehlers-Danlos syndrome type VIIC, a recessively inherited connective-tissue disorder. Alternative splicing results in two transcript variants. The short transcript encodes a protein which has no significant procollagen N-peptidase activity.
Host
Rabbit
Immunogen
Synthetic peptide conjugated to KLH
Isotype
IgG
Quantity
100 µl
Reactivity
Human
Recombinant
FALSE
Regulatory
RUO
Shipping Condition
Ice Packs
Buffer
PBS, pH 7.4 with 0.02% Sodium Azide
Concentration
100µg/100ul
Description
Specificity: Not tested with other proteinsRelevance: ADAMTS2 is a member of the larger family of ADAMs (A Disintegrin And Metalloproteinase) metalloproteinases containing thrombospondin (TS) repeats. ADAMTS2 (A Disintegrin And Metalloproteinase with ThromboSpondin-2 motif), also known as Procollagen I N-Proteinase (PNP), was first described in calf skin as a proteinase that processes the amino end of Type-I collagen. PNP expression was found in skin, aorta, liver, tendon, bladder, retina, and skeletal muscle. Later, PNP was found to be a member of a larger family of ADAMs metalloproteinases containing thrombospondin (TS) repeats. Full length human ADAMTS2 contains 1211 amino acids (bovine, 1205 amino acids) and has a predicted mass of 134.7 kDa, but glycosylation and the abundance of cysteine residues gives ADAMTS2 a greater apparent molecular weight on reduced SDS-PAGE gels. Purified ADAMTS2 resolves at a lower molecular weight of 107 kDa, due to cleavage at the furin site. ADAMTS2 contains the canonical HexxHxxxxxH zinc metalloproteinase motif, and has been shown to be proteolytically active, cleaving procollagen. In addition to the metalloprotease domain, ADAMTS2 has a propeptide domain, a prohormone convertase (PC, furin) cleavage site, a cysteine-rich domain, and three thrombospondin 1 like domains, followed by a unique C-terminal domain. ADAMTS2 does not have a transmembrane domain, unlike many of the ADAMs proteases, and is a secreted protein, much of which binds to the ECM (extracellular matrix). ADAMTS2 knockout mice develop fragile skin (similar to dermatospaxis), and male infertility. Mutations of the ADAMTS2 gene are responsible for human Ehlers-Danlos syndrome type VII C and bovine dermatosparaxis. ADAMTS2 is involved in collagen biosynthesis and may also play role in development and angiogenesis.
Format
Liquid
Purity
Purified
Storage
This product is stable for several weeks at 4°C as an undiluted liquid. Dilute only prior to immediate use. For extended storage, aliquot contents and freeze at -20°C or below. Avoid cycles of freezing and thawing. Expiration date is one (1) year from date of receipt.
Storage Condition
-20C
Applications
WB, ELISA
Description
Recommended dilutions Peptide ELISA: 1:160,000 . WB: 1/500 - 1/1000. Not yet tested in other applications. Optimal dilutions/concentrations should be determined by the end user, WB ; ELISA