The ADAMTSL2 Polyclonal Antibody (PAC015315) is a valuable tool for researchers studying ADAMTSL2, an extracellular matrix protein involved in tissue development and maintenance. This antibody, produced in rabbits, exhibits high reactivity with human samples and has been validated for use in Western blot applications. By targeting the ADAMTSL2 protein, this antibody enables accurate detection and analysis in a variety of cell types, making it an ideal choice for investigations in developmental biology and disease research.ADAMTSL2 is known to play a crucial role in regulating tissue growth and integrity, making it a key player in processes such as collagen fibril organization and elastic fiber assembly.
Its function in maintaining tissue structure and function makes it a promising target for research into conditions like connective tissue disorders, cardiovascular diseases, and skeletal abnormalities. Understanding the role of ADAMTSL2 can provide valuable insights into disease mechanisms and potential therapeutic interventions aimed at promoting tissue homeostasis and repair.
Antibody Name:
ADAMTSL2 Antibody (PACO15315)
Antibody SKU:
PACO15315
Size:
50ul
Host Species:
Rabbit
Tested Applications:
ELISA, WB
Recommended Dilutions:
ELISA:1:1000-1:2000, WB:1:200-1:1000
Species Reactivity:
Human, Mouse
Immunogen:
Fusion protein of human ADAMTSL2
Form:
Liquid
Storage Buffer:
-20°C, pH7.4 PBS, 0.05% NaN3, 40% Glycerol
Purification Method:
Antigen affinity purification
Clonality:
Polyclonal
Isotype:
IgG
Conjugate:
Non-conjugated
Gel: 8%SDS-PAGE, Lysate: 40 μg, Lane 1-2: K562 cells, Jurkat cells, Primary antibody: PACO15315(ADAMTSL2 Antibody) at dilution 1/350, Secondary antibody: Goat anti rabbit IgG at 1/8000 dilution, Exposure time: 5 minutes.
Background:
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 β binding protein 1. Mutations in this gene have been associated with geleophysic dysplasia.
Synonyms:
ADAMTS-like 2
UniProt Protein Function:
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, signal peptide; Protease; Secreted; Extracellular matrixChromosomal Location of Human Ortholog: 9q34.2Cellular Component: proteinaceous extracellular matrixMolecular Function: protein binding; zinc ion binding; metalloendopeptidase activityBiological Process: proteolysis; negative regulation of transforming growth factor beta receptor signaling pathwayDisease: Geleophysic Dysplasia 1
UniProt Protein Details:
NCBI Summary:
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]