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MABN2427 Anti-polyQ specific Antibody, clone MW1

MABN2427
100 μg  
Purchase on Sigma-Aldrich

概述

Replacement Information

重要规格表

Species ReactivityKey ApplicationsHostFormatAntibody Type
M, H, RICC, IHC, WBMPurifiedMonoclonal Antibody
Description
Catalogue NumberMABN2427
DescriptionAnti-polyQ specific Antibody, clone MW1
Alternate Names
  • Htt mutant
  • PolyQ-Htt
  • Clone MW1
  • Atrophin-1
Background InformationHuntington disease (HD) is a neurodegenerative disorder caused by an expansion of a polyglutamine (PolyQ) domain in the protein huntingtin (htt) that leads to its aggregation into fibrils. PolyQ expansion above 35 -40 results in disease associated with htt aggregation into inclusion bodies. These expanded PolyQ repeats adopt multiple potentially toxic conformations increase atrophin-1 and huntingtin levels and abnormally sequester proteins that are essential for transcription. PolyQ domains of different lengths can display different conformations. The primary sites of neuropathology show variations between different PolyQ disorders, but usually include the cerebellum, striatum, cerebral cortex, brainstem, spinal cord, and thalamus. Huntington s disease (HD) and dentatorubral pallidoluysian atrophy (DRPLA) display clinical similarities, but exhibit different regional pathologies. For example, in HD striatum is the most affected region whereas in DRPLA dentate nucleus of the cerebellum is more severely affected. Clone MW1 strongly binds to the expanded PolyQ of mutant Htt, but does not display any detectible binding to normal human or wild-type murine Htt. The clone MW1, which bind to the polyQ repeat in htt is reported to increase htt-induced toxicity and aggregation. (The clone MW1, which bind to the polyQ repeat in htt is reported to increased htt-induced toxicity and aggregation. (Ref.: Luthi-Carter, R et al. (2012). Hum. Mol. Gen. 11(17); 1927-1937; Legleiter, J et al. (2009). J. Biol. Chem. 284(32); 21647-21658; Ko, J et al. (2001). Brain Res. Bull. 56 (3/4); 319-329).
References
Product Information
FormatPurified
PresentationPurified mouse monoclonal antibody IgG2b in buffer containing 0.1 M Tris-Glycine (pH 7.4), 150 mM NaCl with 0.05% sodium azide.
Quality LevelMQ100
Applications
ApplicationAnti-polyQ specific, clone MW1, Cat. No. MABN2427, is a highly specific mouse monoclonal antibody that targets expanded PolyQ repeats from Huntingtin (Htt) and has been tested for use in Immunocytochemistry, Immunohistochemistry, and Western Blotting.
Key Applications
  • Immunocytochemistry
  • Immunohistochemistry
  • Western Blotting
Application NotesWestern Blotting Analysis: A representative lot detected expanded polyQ repeats in Htt in Western Blotting applications (Ko, J., et. al. (2001). Brain Res Bull. 56(3-4):319-29; Legleiter, J., et. al. (2009). J Biol Chem. 284(32):21647-58).

Immunohistochemistry Analysis: A representative lot detected expanded polyQ repeats in Immunohistochemistry applications (Ko, J., et. al. (2001). Brain Res Bull. 56(3-4):319-29).

Immunocytochemistry Analysis: A representative lot detected expanded polyQ repeats in Immunocytochemistry applications (Legleiter, J., et. al. (2009). J Biol Chem. 284(32):21647-58).
Biological Information
ImmunogenGST-tagged recombinant proteins expressed from two constructs containing the polyQ domain (19 repeats) and 34 amino acids of the dentatorubralpalliodoluysian atrophy (DRPLA) gene.
CloneMW1
ConcentrationPlease refer to lot specific datasheet.
HostMouse
SpecificityClone MW1 specifically recognize the polyQ domain and does not react with wild-type Htt in any significant manner.
IsotypeIgG2b
Species Reactivity
  • Mouse
  • Human
  • Rat
Species Reactivity NoteHuman, Mouse, Rat
Antibody TypeMonoclonal Antibody
Entrez Gene Number
Purification MethodProtein G purified
UniProt Number
Molecular Weight~350 kDa observed. Uncharacterized bands may be observed in some lysate(s).
Physicochemical Information
Dimensions
Materials Information
Toxicological Information
Safety Information according to GHS
Safety Information
Product Usage Statements
Quality AssuranceEvaluated by Western Blotting in mouse brain tissue lysate.

Western Blotting Analysis: 0.5 µg/mL of this antibody detected expanded polyQ repeats in Htt in 10 µg of mouse brain tissue lysate. It does not react with normal Htt.
Usage Statement
  • Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.
Storage and Shipping Information
Storage ConditionsStable for 1 year at 2-8°C from date of receipt.
Packaging Information
Material Size100 μg
Transport Information
Supplemental Information
Specifications
Global Trade Item Number
产品目录编号 GTIN
MABN2427 04054839399794

Documentation

Anti-polyQ specific Antibody, clone MW1 分析证书

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