Anti-ADAMTS2 Antibody

For Research Use Only. Not for use in diagnostic procedures.

Polyclonal

IgG

Rabbit

Purified

Liquid

100ug/100ul (lot specific)

This product is stable for several weeks at 4 degree C as an undiluted liquid. Dilute only prior to immediate use. For extended storage, aliquot contents and freeze at -20 degree C or below. Avoid cycles of freezing and thawing. Expiration date is one (1) year from date of receipt.

Small volumes of anti-ADAMTS2 antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

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.

Peptide ELISA (EIA), Western Blot (WB)

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.

61,756 Da

ADAM metallopeptidase with thrombospondin type 1 motif, 2

A disintegrin and metalloproteinase with thrombospondin motifs 2; a disintegrin-like and metalloprotease (reprolysin type) with thrombospondin type 1 motif, 2; procollagen I N-proteinase; procollagen I/II amino propeptide-processing enzyme; procollagen N-endopeptidase

A disintegrin and metalloproteinase with thrombospondin motifs 2

ATS2_HUMAN

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 multiple transcript variants. [provided by RefSeq, Jul 2010]

ADAMTS2: Cleaves the propeptides of type I and II collagen prior to fibril assembly. Does not act on type III collagen. May also play a role in development that is independent of its role in collagen biosynthesis. Defects in ADAMTS2 are the cause of Ehlers-Danlos syndrome type 7C (EDS7C). EDS is a connective tissue disorder characterized by hyperextensible skin, atrophic cutaneous scars due to tissue fragility and joint hyperlaxity. EDS7C is marked by extremely fragile tissues, hyperextensible skin and easy bruising. Facial skin contains numerous folds, as in the cutis laxa syndrome. 2 isoforms of the human protein are produced by alternative splicing.

Protein type: Secreted; EC 3.4.24.14; Extracellular matrix; Secreted, signal peptide; Motility/polarity/chemotaxis; Protease

Chromosomal Location of Human Ortholog: 5qter

Cellular Component: proteinaceous extracellular matrix; extracellular region

Molecular Function: metallopeptidase activity; zinc ion binding; metalloendopeptidase activity

Biological Process: skin development; collagen catabolic process; extracellular matrix organization and biogenesis; collagen fibril organization; protein processing; spermatogenesis; proteolysis; lung development

Disease: Ehlers-danlos Syndrome, Type Vii, Autosomal Recessive

Colige,A., Nuytinck,L., Hausser,I.,et al. Novel types of mutation responsible for the dermatosparactic type of Ehlers-Danlos syndrome (Type VIIC) and common polymorphisms in the ADAMTS2 gene.J. Invest. Dermatol. 123 (4), 656-663 (2004

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