ADAMTSL2; ADAMTS-like 2; FLJ45164; KIAA0605; OTTHUMP00000064620; OTTHUMP00000162492; ADAMTSL2 antibody; ADAMTS-like 2 antibody; FLJ45164 antibody; KIAA0605 antibody; OTTHUMP00000064620 antibody; OTTHUMP00000162492 antibody

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

Polyclonal

Goat

Purified from goat serum by ammonium sulphate precipitation followed by antigen affinity chromatography using the immunizing peptide.

Supplied at 0.5 mg/ml in Tris saline, 0. 02% sodium azide, pH7.3 with 0.5% bovine serum albumin.

100ug specific antibody in 200ul (lot specific)

Aliquot and store at -20 degree C. Minimize freezing and thawing.

Manufactured in an ISO 9001:2008 Certified Laboratory.

Small volumes of anti-ADAMTSL2 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.

Peptide ELISA (EIA)

Peptide ELISA: Antibody detection limit dilution 1: 128000.
Western Blot: Preliminary experiments gave an approx 25kDa band in Human Kidney, Liver and Lung lysates after 0.1ug/ml antibody staining. Please note that currently we cannot find an explanation in the literature for the band we observe given the calculated size of 105kDa according to NP_055509.2. The 25kDa band was successfully blocked by incubation with the immunizing peptide.

104,621 Da

ADAMTS like 2

ADAMTS-like protein 2

ADAMTS-like protein 2

KIAA0605; ADAMTSL-2??[Similar Products]

ATL2_HUMAN

This gene encodes a member of the ADAMTS (a disintegrin and metalloproteinase with thrombospondin motifs) and ADAMTS-like 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 protein encoded by this gene lacks the protease domain, and is therefore of a member of the the ADAMTS-like protein subfamily. It is a secreted glycoprotein that binds the cell surface and extracellular matrix; it also interacts with latent transforming growth factor beta binding protein 1. Mutations in this gene have been associated with geleophysic dysplasia. [provided by RefSeq, Feb 2009]

ADAMTSL2: Defects in ADAMTSL2 are the cause of geleophysic dysplasia type 1 (GPHYSD1). An autosomal recessive disorder characterized by severe short stature, short hands and feet, joint limitations, and skin thickening. Radiologic features include delayed bone age, cone-shaped epiphyses, shortened long tubular bones, and ovoid vertebral bodies. Affected individuals have characteristic facial features including a 'happy' face with full cheeks, shortened nose, hypertelorism, long and flat philtrum, and thin upper lip. Other distinctive features include progressive cardiac valvular thickening often leading to an early death, toe walking, tracheal stenosis, respiratory insufficiency, and lysosomal-like storage vacuoles in various tissues.

Protein type: Extracellular matrix; Secreted, signal peptide; Secreted; Protease

Chromosomal Location of Human Ortholog: 9q34.2

Cellular Component: proteinaceous extracellular matrix

Molecular Function: metalloendopeptidase activity; microfibril binding; protein binding; zinc ion binding

Biological Process: extracellular matrix organization and biogenesis; negative regulation of transforming growth factor beta receptor signaling pathway; proteolysis

Disease: Geleophysic Dysplasia 1

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