MAB2166 Anti-Huntingtin Protein Antibody, a.a. 181-810, clone 1HU-4C8

MAB2166
100 µL  
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      Overview

      Replacement Information

      Key Specifications Table

      Species ReactivityKey ApplicationsHostFormatAntibody Type
      H, M, R, Mk, Ht, Rb ELISA, ICC, IH(P), IP, WB M Ascites Monoclonal Antibody
      Description
      Catalogue NumberMAB2166
      Brand Family Chemicon®
      Trade Name
      • Chemicon
      DescriptionAnti-Huntingtin Protein Antibody, a.a. 181-810, clone 1HU-4C8
      Alternate Names
      • Huntingtin
      • Huntington's Disease Protein
      • HD Protein
      Background InformationHuntington disease (HD) is a hereditary, progressive, neurodegenerative ailment characterized by personality changes, motor impairment and subcortical dementia. The molecular basis of the disease involves the expansion of the trinucleotide CAG, coding for polyglutamine in the first exon of a chromosome four gene (4p16.3), which normally produces a widely expressed 3136 a.a. (~350 kDa) protein huntingtin with unclear function. The protein is found in the perinuclear region along with microtubules, and in the centrosomal region along with gamma-tubulin. Huntingtin is necessary for neuronal survival and is involved in synaptic vesicle trafficking, microtubule binding and may also have a role in apoptosis. In the HD condition, neuronal cells with the mutant form of huntingtin possess intranuclear aggregations of the N-terminal fragment, causing damaging inclusions in perinuclear locations and striatal neuron cell death. Wild-type huntington and anti-huntingtin reduce aggregation and cellular toxicity of the mutant huntingtin form in mammalian cell models of HD. Huntingtin is known to interact with GAPDH, HAP-1, SP1 and TAFII130.
      References
      Product Information
      FormatAscites
      Control
      • Normal human cerebral cortex lysate
      PresentationAscites mouse monoclonal IgG1κ liquid containing no preservative
      Applications
      ApplicationAnti-Huntingtin Protein Antibody, a.a. 181-810, clone 1HU-4C8 is an antibody against Huntingtin Protein for use in ELISA, IC, IH(P), IP & WB.
      Key Applications
      • ELISA
      • Immunocytochemistry
      • Immunohistochemistry (Paraffin)
      • Immunoprecipitation
      • Western Blotting
      Application NotesELISA:
      A 1:500-1:5,000 dilution of a previous lot was used on ELISA.

      Immunohistochemistry:
      A 1:500-1:5,000 dilution from a previous lot was used on frozen and microwave oven treated paraffin sections (human tissue).

      Immunocytochemistry:
      1:500-1:5,000 on a previous lot was used on transfected cells.

      Immunoprecipitation:
      A 1:500-1:5,000 dilution of a previous lot was used on immunoprecipitation.

      Western blot:
      1:500-1:5,000. Should detect a band migrating at approximately 350-400 kDa by Western blot (Nature Genetics 10:104-110.).

      Optimal working dilutions must be determined by the end user.
      Biological Information
      ImmunogenHuntingtin fragment from a.a. 181 to 810 as a fusion protein.
      Epitopea.a. 181-810
      Clone1HU-4C8
      ConcentrationPlease refer to the Certificate of Analysis for the lot-specific concentration.
      HostMouse
      SpecificityHuntingtin Protein. No detectable cross reactivity to other proteins by Western blot.
      IsotypeIgG1κ
      Species Reactivity
      • Human
      • Mouse
      • Rat
      • Monkey
      • Hamster
      • Rabbit
      Species Reactivity NoteRat. Expected to react with human, monkey, hamster and mouse based on sequence homology
      Antibody TypeMonoclonal Antibody
      Entrez Gene Number
      Entrez Gene SummaryHuntingtin is a disease gene linked to Huntington's disease, a neurodegenerative disorder characterized by loss of striatal neurons. This is thought to be caused by an expanded, unstable trinucleotide repeat in the huntingtin gene, which translates as a polyglutamine repeat in the protein product. A fairly broad range in the number of trinucleotide repeats has been identified in normal controls, and repeat numbers in excess of 40 have been described as pathological. The huntingtin locus is large, spanning 180 kb and consisting of 67 exons. The huntingtin gene is widely expressed and is required for normal development. It is expressed as 2 alternatively polyadenylated forms displaying different relative abundance in various fetal and adult tissues. The larger transcript is approximately 13.7 kb and is expressed predominantly in adult and fetal brain whereas the smaller transcript of approximately 10.3 kb is more widely expressed. The genetic defect leading to Huntington's disease may not necessarily eliminate transcription, but may confer a new property on the mRNA or alter the function of the protein. One candidate is the huntingtin-associated protein-1, highly expressed in brain, which has increased affinity for huntingtin protein with expanded polyglutamine repeats. This gene contains an upstream open reading frame in the 5' UTR that inhibits expression of the huntingtin gene product through translational repression
      Gene Symbol
      • HTT
      • IT15
      • SLC6A4
      • OCD1
      • HD
      Purification MethodUnpurified
      UniProt Number
      UniProt SummaryFUNCTION: SwissProt: P42858 # May play a role in microtubule-mediated transport or vesicle function.
      SIZE: 3144 amino acids; 347860 Da
      SUBUNIT: Binds SH3GLB1 (By similarity). Interacts through its N- terminus with FNBP3. Interacts with PQBP1, SETD2 and SYVN.
      SUBCELLULAR LOCATION: Cytoplasm. Nucleus.
      TISSUE SPECIFICITY: Widely expressed with the highest level of expression in the brain (nerve fibers, varicosities, and nerve endings). In the brain, the regions where it can be mainly found are the cerebellar cortex, the neocortex, the striatum, and the hippocampal formation.
      PTM: Cleaved by apopain downstream of the polyglutamine stretch. The resulting amino-terminal fragment is cytotoxic and provokes apoptosis. & Forms with expanded polyglutamine expansion are specifically ubiquitinated by SYVN1, which promotes their proteasomal degradation.
      DISEASE: SwissProt: P42858 # Defects in HD are the cause of Huntington disease (HD) [MIM:143100]. HD is an autosomal dominant neurodegenerative disorder characterized by involuntary movements (chorea), general motor impairment, psychiatric disorders and dementia. Onset of the disease occurs usually in the third or fourth decade of life and symptoms progressively worsen leading to death in 10 to 20 years. Onset and clinical course depend on the degree of poly-Gln repeat expansion, longer expansions resulting in earlier onset and more severe clinical manifestations. HD affects 1 in 10,000 individuals of European origin. Neuropathology of Huntington disease displays a distinctive pattern with loss of neurons, especially in the caudate and putamen (striatum).
      SIMILARITY: SwissProt: P42858 ## Belongs to the hungtintin family. & Contains 10 HEAT repeats.
      Molecular Weight~ 350-400 kDa
      Physicochemical Information
      Dimensions
      Materials Information
      Toxicological Information
      Safety Information according to GHS
      Safety Information
      Product Usage Statements
      Quality AssuranceRoutinely evaluated by Western Blot on rat brain lysates.

      Western Blot Analysis:
      1:1000 dilution of this lot detected huntingtin protein on 10 μg of rat brain lysates.
      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 -20ºC in undiluted aliquots 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

      Documentation

      SDS

      Title

      Safety Data Sheet (SDS) 

      Certificates of Analysis

      TitleLot Number
      Anti-Huntingtin Protein, a.a. 181-810, -26891472689147
      Anti-Huntingtin Protein, a.a. 181-810, -27806312780631
      Anti-Huntingtin Protein, a.a. 181-810, -28196872819687
      Anti-Huntingtin Protein, a.a. 181-810, clone 1HU-4C82474858
      Anti-Huntingtin Protein, a.a. 181-810, clone 1HU-4C83068437
      Anti-Huntingtin Protein, a.a. 181-810, clone 1HU-4C8 - 21224232122423
      Anti-Huntingtin Protein, a.a. 181-810, clone 1HU-4C8 - 23665252366525
      Anti-Huntingtin Protein, a.a. 181-810, clone 1HU-4C8 - 23888242388824
      Anti-Huntingtin Protein, a.a. 181-810, clone 1HU-4C8 - 19732141973214
      Anti-Huntingtin Protein, a.a. 181-810, clone 1HU-4C8 - 20159712015971

      References

      Reference overviewApplicationSpeciesPub Med ID
      A striatal-enriched intronic GPCR modulates huntingtin levels and toxicity.
      Yao, Y; Cui, X; Al-Ramahi, I; Sun, X; Li, B; Hou, J; Difiglia, M; Palacino, J; Wu, ZY; Ma, L; Botas, J; Lu, B
      eLife  4  2015

      Show Abstract
      25738228 25738228
      Effects on murine behavior and lifespan of selectively decreasing expression of mutant huntingtin allele by supt4h knockdown.
      Cheng, HM; Chern, Y; Chen, IH; Liu, CR; Li, SH; Chun, SJ; Rigo, F; Bennett, CF; Deng, N; Feng, Y; Lin, CS; Yan, YT; Cohen, SN; Cheng, TH
      PLoS genetics  11  e1005043  2015

      Show Abstract
      25760041 25760041
      Scalable production in human cells and biochemical characterization of full-length normal and mutant huntingtin.
      Huang, B; Lucas, T; Kueppers, C; Dong, X; Krause, M; Bepperling, A; Buchner, J; Voshol, H; Weiss, A; Gerrits, B; Kochanek, S
      PloS one  10  e0121055  2015

      Show Abstract
      25799558 25799558
      Huntington disease iPSCs show early molecular changes in intracellular signaling, the expression of oxidative stress proteins and the p53 pathway.
      Szlachcic, WJ; Switonski, PM; Krzyzosiak, WJ; Figlerowicz, M; Figiel, M
      Disease models & mechanisms  8  1047-57  2015

      Show Abstract
      Western Blotting26092128 26092128
      Ultrasensitive measurement of huntingtin protein in cerebrospinal fluid demonstrates increase with Huntington disease stage and decrease following brain huntingtin suppression.
      Southwell, AL; Smith, SE; Davis, TR; Caron, NS; Villanueva, EB; Xie, Y; Collins, JA; Ye, ML; Sturrock, A; Leavitt, BR; Schrum, AG; Hayden, MR
      Scientific reports  5  12166  2015

      Show Abstract
      26174131 26174131
      Nuclear retention of full-length HTT RNA is mediated by splicing factors MBNL1 and U2AF65.
      Sun, X; Li, PP; Zhu, S; Cohen, R; Marque, LO; Ross, CA; Pulst, SM; Chan, HY; Margolis, RL; Rudnicki, DD
      Scientific reports  5  12521  2015

      Show Abstract
      Western Blotting26218986 26218986
      Identification of binding sites in Huntingtin for the Huntingtin Interacting Proteins HIP14 and HIP14L.
      Sanders, SS; Mui, KK; Sutton, LM; Hayden, MR
      PloS one  9  e90669  2014

      Show Abstract
      24651384 24651384
      Polyglutamine- and temperature-dependent conformational rigidity in mutant huntingtin revealed by immunoassays and circular dichroism spectroscopy.
      Fodale, V; Kegulian, NC; Verani, M; Cariulo, C; Azzollini, L; Petricca, L; Daldin, M; Boggio, R; Padova, A; Kuhn, R; Pacifici, R; Macdonald, D; Schoenfeld, RC; Park, H; Isas, JM; Langen, R; Weiss, A; Caricasole, A
      PloS one  9  e112262  2014

      Show Abstract
      25464275 25464275
      Quantification assays for total and polyglutamine-expanded huntingtin proteins.
      Macdonald, D; Tessari, MA; Boogaard, I; Smith, M; Pulli, K; Szynol, A; Albertus, F; Lamers, MB; Dijkstra, S; Kordt, D; Reindl, W; Herrmann, F; McAllister, G; Fischer, DF; Munoz-Sanjuan, I
      PloS one  9  e96854  2014

      Show Abstract
      24816435 24816435
      A unique four-hub protein cluster associates to glioblastoma progression.
      Simeone, P; Trerotola, M; Urbanella, A; Lattanzio, R; Ciavardelli, D; Di Giuseppe, F; Eleuterio, E; Sulpizio, M; Eusebi, V; Pession, A; Piantelli, M; Alberti, S
      PloS one  9  e103030  2014

      Show Abstract
      25050814 25050814

      Data Sheet

      Title
      Anti-Huntingtin Protein, a.a. 181-810, clone 1HU-4C8 - Data Sheet

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      Categories

      Life Science Research > Antibodies and Assays > Primary Antibodies