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MABN2450-25UL Anti-mutant HTT Antibody, clone PHP2

Anti-mutant HTT, clone PHP2, Cat. No. MABN2450, is a highly specific mouse monoclonal antibody that targets Huntingtin and has been tested for use in Dot Blot, Electron Microscopy, Immunohistochemistry, Inhibition/Function Analysis, and Western Blotting.

MABN2450-25UL
25 µL  
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      概要

      Replacement Information
      Description
      Catalogue NumberMABN2450-25UL
      DescriptionAnti-mutant HTT Antibody, clone PHP2
      Alternate Names
      • Huntingtin
      • Huntington disease protein
      • HD protein
      Background InformationHuntingtin (UniProt: P42858; also known as Huntington disease protein, HD protein) is encoded by the HTT (also known as HD, IT15) gene (Gene ID: 3064) in human. The protein is found in the perinuclear region along with microtubules, and in the centrosomal region along with gamma-tubulin. It is expressed in the brain and is mainly found in the cerebellar cortex, the neocortex, the striatum, and the hippocampal formation. Huntingtin is necessary for neuronal survival and is involved in synaptic vesicle trafficking, microtubule binding and may also have a role in apoptosis. It plays a role in microtubule-mediated transport and vesicle function. Huntingtin protein contains a nuclear export signal (aa 2395-2404) and 5 HEAT (Huntington, Elongation Factor 3, PR65/A, TOR) domains. Huntingtin protein is cleaved by apopain downstream of the polyglutamine stretch and the resulting N-terminal fragment is cytotoxic and provokes apoptosis. Phosphorylation at Serine 1179 and 1199 by CDK5 in response to DNA damage in nuclei of neurons protects neurons against polyglutamine expansion as well as DNA damage mediated toxicity. The N-terminal fragments of mutant huntingtin (mHTT) misfold and assemble into oligomers that ultimately bundle into insoluble fibrils. Misfolding and self-aggregation of specific proteins is commonly observed in well-known neurodegenerative diseases, including Huntington s disease (HD), Alzheimer s disease (AD), Parkinson s disease (PD), and amyotrophic lateral sclerosis (AML). Abnormal polyglutamine (polyQ) repeat sequence or stretch result in protein misfolding and neurodegeneration. Mutations in HTT gene cause Huntington disease that is characterized by involuntary movements (chorea), general motor impairment, psychiatric disorders and dementia. Wild-type huntingtin and anti-huntingtin antibody reduces aggregation and cellular toxicity of the mutant huntingtin form in mammalian cell models of Huntington disease. Clone PHP2 reacts with the peptide sequence QAQPLLPQP within the proline rich domain (PRD) of HTT. It is also shown to block seeding and subsequent fibril assembly of mHTTx1 in cell culture models of HD. (Ref.: Ko, J., et al. (2018). Hum. Mol. Gen. 27(13); 2330-2343).
      References
      Product Information
      FormatPurified
      PresentationPurified mouse monoclonal antibody IgG2a in PBS without azide.
      Applications
      ApplicationAnti-mutant HTT, clone PHP2, Cat. No. MABN2450, is a highly specific mouse monoclonal antibody that targets Huntingtin and has been tested for use in Dot Blot, Electron Microscopy, Immunohistochemistry, Inhibition/Function Analysis, and Western Blotting.
      Key Applications
      • Dot Blot
      • Electron Microscopy
      • Immunohistochemistry
      • Inhibits Activity/Function
      • Western Blotting
      Application NotesWestern Blotting Analysis: A 1:1,000 dilution from a representative lot detected mutant HTT in Brain tissue lysates of 6 or 9 month-old WT or Q140 KI HD mice (Courtesy of Ali Khoshnan, Ph.D., California Institute of Technology, Paadena, CA).

      Inhibits Activity/Function Analysis: A representative lot blocked seeding and subsequent fibril assembly of mHTTx1 in vitro and in a cell culture model of HD. (Ko, J., et. al. (2018). Hum. Mol. Genet. 27(13):2330-2343).

      Electron Microscopy Analysis: A representative lot detected mutant HTT in Immuno-Electron Microscopy applications (Ko, J., et. al. (2018). Hum. Mol. Genet. 27(13): 2330-2343).

      Dot Blot Analysis: A representative lot detected mutant HTT in Dot Blot applications (Ko, J., et. al. (2018). Hum. Mol. Genet. 27(13): 2330-2343).

      Immunohistochemistry Analysis: A representative lot detected mutant HTT in Immunohistochemistry applications (Ko, J., et. al. (2018). Hum. Mol. Genet. 27(13): 2330-2343).

      Western Blotting Analysis: A representative lot detected mutant HTT in Western Blotting applications (Ko, J., et. al. (2018). Hum. Mol. Genet. 27(13): 2330-2343).
      Biological Information
      ImmunogenGST-tagged recombinant exon1 of wild-type human huntingtin HTTx1 (20Q) protein.
      ClonePHP2
      ConcentrationPlease refer to lot specific datasheet.
      HostMouse
      SpecificityClone PHP1 is mouse monoclonal antibody that detects mutated Huntingtin protein. It targets an epitope QAQPLLPQP within the proline-rich domain.
      IsotypeIgG2aκ
      Species Reactivity
      • Human
      • Mouse
      Species Reactivity NoteHuman, Mouse.
      Antibody TypeMonoclonal Antibody
      Entrez Gene Number
      Gene Symbol
      • HTT
      • HD
      • IT15
      Purification MethodProtein G purified
      UniProt Number
      Molecular Weight347.60 kDa calculated.
      Physicochemical Information
      Dimensions
      Materials Information
      Toxicological Information
      Safety Information according to GHS
      Safety Information
      Product Usage Statements
      Quality AssuranceIsotype testing: Identity Confirmation by Isotyping Test.

      Isotyping Analysis: The identity of this monoclonal antibody is confirmed by isotyping test to be mouse IgG2a.
      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 -20°C from date of receipt. Handling Recommendations: Upon receipt and prior to removing the cap, centrifuge the vial and gently mix the solution. Aliquot into microcentrifuge tubes and store at -20°C. Avoid repeated freeze/thaw cycles, which may damage IgG and affect product performance.
      Packaging Information
      Material Size25 µL
      Transport Information
      Supplemental Information
      Specifications
      Global Trade Item Number
      カタログ番号 GTIN
      MABN2450-25UL 04061841235972

      Documentation

      Anti-mutant HTT Antibody, clone PHP2 (M)SDS

      タイトル

      英語版製品安全データシート（(M)SDS） 

      Anti-mutant HTT Antibody, clone PHP2 試験成績書（CoA）

      タイトルロット番号
      Anti-mutant HTT, clone PHP2 - Q3329768 Q3329768

      参考資料

      参考資料の概要Pub Med ID
      Identification of distinct conformations associated with monomers and fibril assemblies of mutant huntingtin.
      Ko, J; Isas, JM; Sabbaugh, A; Yoo, JH; Pandey, NK; Chongtham, A; Ladinsky, M; Wu, WL; Rohweder, H; Weiss, A; Macdonald, D; Munoz-Sanjuan, I; Langen, R; Patterson, PH; Khoshnan, A
      Hum Mol Genet  27  2330-2343  2018

      概要を表示する       		29912367 29912367