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Invitrogen™ EXT1 Polyclonal Antibody
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Catalog No. PIPA5112922
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100 μg
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PIPA5112922 100 μg
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Catalog No. PIPA5112922 Supplier Invitrogen™ Supplier No. PA5112922
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Description

Rabbit Polyclonal Antibody

EXT1 Polyclonal Antibody for Western Blot, ELISA

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.
TRUSTED_SUSTAINABILITY

Specifications

Antigen EXT1
Applications ELISA, Western Blot
Classification Polyclonal
Concentration 1 mg/mL
Conjugate Unconjugated
Formulation PBS with 50% glycerol and 0.03% ProClin 300; pH 7.4
Gene EXT1
Gene Accession No. Q16394
Gene Alias AA409028; exostoses (multiple) 1; exostosin 1; exostosin glycosyltransferase 1; exostosin-1; EXT; Ext1; Glucuronosyl-N-acetylglucosaminyl-proteoglycan 4-alpha-N- acetylglucosaminyltransferase; glucuronosyl-N-acetylglucosaminyl-proteoglycan/N-acetylglucosaminyl-proteoglycan 4-alpha-N-acetylglucosaminyltransferase; Langer-Giedion syndrome chromosome region; LGCR; LGS; multiple exostoses protein 1; Multiple exostoses protein 1 homolog; N-acetylglucosaminyl-proteoglycan 4-beta-glucuronosyltransferase; putative tumor suppressor protein EXT1; TRPS2; TTV
Gene Symbols EXT1
Host Species Rabbit
Immunogen Recombinant Human Exostosin-1 protein (28-171AA).
Purification Method Protein G
Quantity 100 μg
Regulatory Status RUO
Primary or Secondary Primary
Gene ID (Entrez) 2131
Target Species Human
Content And Storage -20°C or -80°C if preferred
Product Type Antibody
Form Liquid
Isotype IgG
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