Abcam, ab126305, Anti-Ext1 antibody

Size: 100µL
Anti-Ext1 antibody (ab126305) is a rabbit polyclonal antibody detecting Ext1 in IHC-P . Suitable for Human .
Key facts
Host species:Rabbit,
Clonality:Polyclonal,
Isotype:IgG,
Carrier free:No,
Reacts with:Human,
Applications:IHC-PSee reactivity dataSee the reactivity data table below for information on validated species and application combinations.,
Immunogen:Recombinant Fragment Protein within Human EXT1 aa 200-350. The exact immunogen used to generate this antibody is proprietary information.Q16394

Product details:
What is this antibody validated in?
Anti-Ext1 antibody (ab126305) is a rabbit polyclonal antibody and is validated for use in Immunohistochemistry (IHC-P) in Human samples.

Properties and Storage Information:
Form-Liquid, Purification technique-Affinity purification Immunogen, Storage buffer-pH: 7.2Preservative: 0.02% Sodium azideConstituents: PBS, 40% Glycerol (glycerin, glycerine), Shipped at conditions-Blue Ice, Appropriate short-term storage conditions-+4°C, Appropriate long-term storage conditions--20°C, Aliquoting information-Upon delivery aliquot, Storage information-Avoid freeze / thaw cycle

Supplementary Information:
This supplementary information is collated from multiple sources and compiled automatically.
Ext1 also known as Exostosin-1 functions as an enzyme that participates in the glycosyltransferase family. It has a mass of approximately 100 kDa. Ext1 plays a significant mechanical role in catalyzing the elongation of heparan sulfate chains. This process involves the transfer of sugar residues to a growing polysaccharide chain which is important for synthesizing heparan sulfate proteoglycans. Ext1 is expressed in various tissues including the brain kidney and cartilage.
Biological function summary
Ext1 contributes to the biosynthesis of heparan sulfate a significant sulfated polysaccharide in the extracellular matrix and cell surface. Heparan sulfate chains influence cell signaling and interaction processes important for cellular homeostasis development and repair. Ext1 operates as part of an enzyme complex with Ext2 another member of the exostosin family facilitating coordinated activity in heparan sulfate polymerization. This complex ensures the controlled addition of sugar residues necessary for proper chain formation.
Pathways
Ext1 plays a role in the heparan sulfate biosynthesis and the Hedgehog signaling pathway. In the context of heparan sulfate biosynthesis Ext1 modifies the structure of heparan sulfate chains that modulate various signaling pathways influencing processes such as cell proliferation and differentiation. In the Hedgehog pathway Ext1 interacts with proteins like Sonic Hedgehog (SHH) by contributing to the modification of the cellular microenvironment affecting signal transmission.
Ext1 mutations or abnormalities associate with hereditary multiple exostoses (HME) a condition characterized by the growth of exostoses or bone spurs. The defect in Ext1 disrupts normal heparan sulfate synthesis contributing to abnormal skeletal development in HME. Additionally abnormalities in Ext1 exhibit connections to osteochondroma formation. The dysregulation of Ext1 may also influence the activity of other proteins such as Ext2 as these proteins work closely in glycosaminoglycan biosynthesis affecting disease progression and phenotype expression.