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          • Primary Antibodies ›
          • EXT1 Antibodies

          Invitrogen

          EXT1 Polyclonal Antibody

          View all (7) EXT1 antibodies

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          Cite EXT1 Polyclonal Antibody

          Additional Information:
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          EXT1 Polyclonal Antibody

          Product Details

          PA5-76041

          Applications
          Tested Dilution
          Publications

          Immunocytochemistry (ICC/IF)

          1:10-1:100
          -
          Product Specifications

          Species Reactivity

          Human, Mouse

          Host/Isotype

          Rabbit / IgG

          Class

          Polyclonal

          Type

          Antibody

          Immunogen

          KLH conjugated synthetic peptide derived from human EXT1: 651-746.
          3D Epitope / Immunogen

          Conjugate

          Unconjugated Unconjugated Unconjugated

          Form

          Liquid

          Concentration

          1 mg/mL

          Amount

          100 µg

          Purification

          Antigen affinity chromatography

          Storage buffer

          PBS, pH 7.2, with 50% glycerol

          Contains

          0.02% sodium azide

          Storage conditions

          Store at 4°C short term. For long term storage, store at -20°C, avoiding freeze/thaw cycles.

          Shipping conditions

          Ambient (domestic); Wet ice (international)

          RRID

          AB_2719769

          Product Specific Information

          The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen and the purity is > 95% (by SDS-PAGE).

          Target Information

          Hereditary multiple exostoses (HME) is an autosomal dominant disorder characterized by the formation of exostoses (EXT), which are cartilage-capped bony protuberances mainly located on long bones. Two proteins associated with EXT, EXT1 and EXT2, form homo/heteromeric complexes in vivo, which leads to the accumulation of both proteins in the Golgi apparatus. EXT1 and EXT2 are endoplasmic reticulum-localized type II transmembrane glycoproteins that possess, or are tightly associated with, glycosyltransferase activities involved in the polymerization of the glycosaminoglycan, heparan sulfate (HS). EXT2 is a protein that harbors the D-glucuronyl (GlcA) and N-acetyl-D-glucosaminyl (GlcNAc) transferase activities required for biosynthesis of HS. EXT1 rescues defective HS biosynthesis and elevates low GlcA and GlcNAc transferase levels in mutated cells.

          For Research Use Only. Not for use in diagnostic procedures. Not for resale without express authorization.

          References (0)

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          Cite this product

          Bioinformatics

          Protein Aliases: exostoses (multiple) 1; Exostosin glycosyltransferase 1; Exostosin-1; Glucuronosyl-N-acetylglucosaminyl-proteoglycan 4-alpha-N- acetylglucosaminyltransferase; glucuronosyl-N-acetylglucosaminyl-proteoglycan/N-acetylglucosaminyl-proteoglycan 4-alpha-N-acetylglucosaminyltransferase; Heparan sulfate co-polymerase subunit EXT1; Langer-Giedion syndrome chromosome region; Multiple exostoses protein 1; Multiple exostoses protein 1 homolog; N-acetylglucosaminyl-proteoglycan 4-beta-glucuronosyltransferase; putative tumor suppressor protein EXT1; putative tumour suppressor/hereditary multiple exostoses candidate gene; unnamed protein product

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          Gene Aliases: AA409028; EXT; EXT1; LGCR; LGS; TRPS2; TTV

          View more View less

          UniProt ID: (Human) Q16394, (Mouse) P97464

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          Entrez Gene ID: (Human) 2131, (Mouse) 14042

          View more View less

          Function(s)
          protein binding acetylglucosaminyltransferase activity glucuronosyltransferase activity transferase activity transferase activity, transferring glycosyl groups heparan sulfate N-acetylglucosaminyltransferase activity protein homodimerization activity metal ion binding protein heterodimerization activity glucuronosyl-N-acetylglucosaminyl-proteoglycan 4-alpha-N-acetylglucosaminyltransferase activity N-acetylglucosaminyl-proteoglycan 4-beta-glucuronosyltransferase activity glycosyltransferase
          Process(es)
          polysaccharide biosynthetic process cell morphogenesis ossification endochondral ossification blood vessel remodeling chondrocyte differentiation glandular epithelial cell differentiation hematopoietic progenitor cell differentiation hypersensitivity heart field specification chondrocyte hypertrophy endochondral bone growth glycosaminoglycan biosynthetic process protein glycosylation vacuole organization cell adhesion cell surface receptor signaling pathway smoothened signaling pathway gastrulation axon guidance brain development endoderm development mesoderm development regulation of blood pressure cell proliferation fibroblast growth factor receptor signaling pathway response to virus response to light intensity gene expression regulation of tumor necrosis factor-mediated signaling pathway neural crest cell differentiation heparan sulfate proteoglycan biosynthetic process stem cell division antigen processing and presentation optic nerve development olfactory bulb development hair cycle process protein catabolic process proteoglycan biosynthetic process collagen fibril organization heparin metabolic process heparin biosynthetic process BMP signaling pathway hair follicle morphogenesis neuron projection development glomerular basement membrane development cell adhesion mediated by integrin social behavior synaptic transmission, glutamatergic multicellular organism growth chondrocyte proliferation limb joint morphogenesis dendritic cell migration lymphocyte adhesion to endothelial cell of high endothelial venule fluid transport wound healing vasodilation fear response ossification involved in bone maturation cellular response to fibroblast growth factor stimulus cell fate commitment bone resorption embryonic morphogenesis sebaceous gland development chondroitin sulfate proteoglycan metabolic process multicellular organismal water homeostasis leukocyte tethering or rolling sulfation sodium ion homeostasis heart contraction canonical Wnt signaling pathway hematopoietic stem cell differentiation bone morphogenesis endochondral bone morphogenesis cartilage development involved in endochondral bone morphogenesis epithelium development epithelial tube branching involved in lung morphogenesis mesenchyme development smoothened signaling pathway involved in lung development developmental growth involved in morphogenesis sweat gland development hematopoietic stem cell homeostasis motor behavior perichondral bone morphogenesis stomach development protein-containing complex assembly dendrite self-avoidance response to growth factor response to heparin vocalization behavior basement membrane organization cellular response to BMP stimulus glomerular visceral epithelial cell differentiation embryonic skeletal joint development lymphocyte migration into lymphoid organs hematopoietic stem cell migration to bone marrow cellular response to virus bone growth tight junction organization mesenchymal cell differentiation involved in bone development cranial skeletal system development response to leukemia inhibitory factor heparan sulfate proteoglycan biosynthetic process, polysaccharide chain biosynthetic process cellular polysaccharide biosynthetic process
          It has to be done as per old AB suggested Products section.

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