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MABN2450-100UL Anti-mutant HTT Antibody, clone PHP2

MABN2450-100UL
100 µL  
Purchase on Sigma-Aldrich

Overview

Replacement Information
Description
Catalogue NumberMABN2450-100UL
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 Size100 µL
Transport Information
Supplemental Information
Specifications
Global Trade Item Number
Catalog Number GTIN
MABN2450-100UL 04061841235965

Documentation

Anti-mutant HTT Antibody, clone PHP2 SDS

Title

Safety Data Sheet (SDS) 

Anti-mutant HTT Antibody, clone PHP2 Certificates of Analysis

TitleLot Number
Anti-mutant HTT, clone PHP2 - 3525929 3525929
Anti-mutant HTT, clone PHP2 - 3574435 3574435
Anti-mutant HTT, clone PHP2 - 3724613 3724613
Anti-mutant HTT, clone PHP2 - 3739690 3739690
Anti-mutant HTT, clone PHP2 - 3815818 3815818
Anti-mutant HTT, clone PHP2 - 3818028 3818028
Anti-mutant HTT, clone PHP2 - 3862778 3862778
Anti-mutant HTT, clone PHP2 - 3887825 3887825
Anti-mutant HTT, clone PHP2 - 3899509 3899509
Anti-mutant HTT, clone PHP2 - 3901425 3901425

References

Reference overviewPub 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

Show Abstract
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