Rabbit ADAMTSL2 Polyclonal Antibody | anti-ADAMTSL2 antibody

ADAMTSL2 Polyclonal Antibody

Cat. Num. AAA2522398

• Gene Names: ADAMTSL2; GPHYSD1
• Reactivity: Human, Mouse, Rat
• Applications: ELISA, Western Blot
• Purity: Antigen affinity purification
• Synonyms: ADAMTSL2; Polyclonal Antibody; ADAMTSL2 Polyclonal Antibody; GPHYSD1; anti-ADAMTSL2 antibody

• Host: Rabbit
• Reactivity: Human, Mouse, Rat
• Clonality: Polyclonal
• Isotype: IgG
• Purity/Purification: Antigen affinity purification
• Concentration: 0.7mg/mL (varies by lot)
• Sequence Length: 951

• Applicable Applications for anti-ADAMTSL2 antibody: ELISA (EIA), Western Blot (WB)
• Application Notes: WB: 1:200-1:1000
• Immunogen: Recombinant protein of human ADAMTSL2
• Buffer: PBS with 0.05% sodium azide, 50% glycerol, pH7.3
• Preparation and Storage: Store at -20 degree C (regular) and -80 degree C (long term). Avoid freeze / thaw cycles.

Western Blot (WB)

(Western Blot analysis of K562 and Jurkat cell using ADAMTSL2 Polyclonal Antibody at dilution of 1:350)

Related Product Information for anti-ADAMTSL2 antibody

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.

NCBI and Uniprot Product Information

• NCBI GI #: 223718260
• NCBI GeneID: 9719
• NCBI Accession #: NP_001138792.1
• NCBI GenBank Nucleotide #: NM_001145320.1
• Molecular Weight: 104,621 Da
• NCBI Official Full Name: ADAMTS-like protein 2
• NCBI Official Synonym Full Names: ADAMTS-like 2
• NCBI Official Symbol: ADAMTSL2
• NCBI Official Synonym Symbols: GPHYSD1
• NCBI Protein Information: ADAMTS-like protein 2; ADAMTSL-2
• UniProt Protein Name: ADAMTS-like protein 2
• Protein Family: ADAMTS-like protein
• UniProt Gene Name: ADAMTSL2
• UniProt Synonym Gene Names: KIAA0605; ADAMTSL-2
• UniProt Entry Name: ATL2_HUMAN

NCBI Description

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]

Uniprot Description

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

Chromosomal Location of Human Ortholog: 9q34.2

Cellular Component: proteinaceous extracellular matrix

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

Biological Process: proteolysis; negative regulation of transforming growth factor beta receptor signaling pathway

Disease: Geleophysic Dysplasia 1