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Invitrogen™ EXT1 Polyclonal Antibody
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Catalog No. PIPA5116952
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PIPA5116952 100 μL
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Catalog No. PIPA5116952 Supplier Invitrogen™ Supplier No. PA5116952
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Description

Rabbit Polyclonal Antibody

Positive Samples: K-562, SW480, PC-3, Mouse liver, Mouse lung.

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, Immunocytochemistry
Classification Polyclonal
Concentration 1.36 mg/mL
Conjugate Unconjugated
Formulation PBS with 50% glycerol and 0.09% sodium azide; pH 7.3
Gene EXT1
Gene Accession No. P97464, 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 Synthetic Peptide.
Purification Method Affinity chromatography
Quantity 100 μL
Regulatory Status RUO
Primary or Secondary Primary
Gene ID (Entrez) 14042, 2131, 299907
Target Species Human, Mouse, Rat
Content And Storage -20°C, Avoid Freeze/Thaw Cycles
Product Type Antibody
Form Liquid
Isotype IgG
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Safety and Handling

WARNING: Cancer - www.P65Warnings.ca.gov
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