Huntingtin Protein Antibody, a.a. 181-810, clone 1HU-4C8 | EMD Millipore MAB2166 product information

This webpage contains legacy information. The product is either no longer available from the supplier or has been delisted at Labome.

product summary

company name :

EMD Millipore

other brands :

Oncogene Research Products, Calbiochem, Novagen, Merck, Upstate Biotechnology, Chemicon, LINCO, Novabiochem, Guava

product type :

antibody

product name :

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

catalog :

MAB2166

quantity :

100 µL

clonality :

monoclonal

host :

mouse

conjugate :

nonconjugated

clone name :

1HU-4C8

The same clone is also sold as:

Invitrogen: MA3-040

reactivity :

African green monkey, human, mouse, rat

application :

western blot, ELISA, immunohistochemistry, immunocytochemistry, immunoprecipitation, flow cytometry, immunohistochemistry - paraffin section, western blot knockout validation

citations: 164

Published Application/Species/Sample/Dilution	Reference
western blot knockout validation; mouse; loading ...; fig 1b	Wang G, Liu X, Gaertig M, Li S, Li X. Ablation of huntingtin in adult neurons is nondeleterious but its depletion in young mice causes acute pancreatitis. Proc Natl Acad Sci U S A. 2016;113:3359-64 pubmed publisher
western blot; mouse; 1:1000; loading ...; fig s1d	Burrus C, McKinstry S, Kim N, Ozlu M, Santoki A, Fang F, et al. Striatal Projection Neurons Require Huntingtin for Synaptic Connectivity and Survival. Cell Rep. 2020;30:642-657.e6 pubmed publisher
western blot; human; 1:500; loading ...; fig 1g ELISA; mouse; 4 ug/ml; loading ...; fig 4b	Zeitler B, Froelich S, Marlen K, Shivak D, Yu Q, Li D, et al. Allele-selective transcriptional repression of mutant HTT for the treatment of Huntington's disease. Nat Med. 2019;25:1131-1142 pubmed publisher
immunocytochemistry; human; 1:500; loading ...; fig 4b	Vidoni C, Secomandi E, Castiglioni A, Melone M, Isidoro C. Resveratrol protects neuronal-like cells expressing mutant Huntingtin from dopamine toxicity by rescuing ATG4-mediated autophagosome formation. Neurochem Int. 2018;117:174-187 pubmed publisher
western blot; mouse; fig s5e	Gómez Pastor R, Burchfiel E, Neef D, Jaeger A, Cabiscol E, McKinstry S, et al. Abnormal degradation of the neuronal stress-protective transcription factor HSF1 in Huntington's disease. Nat Commun. 2017;8:14405 pubmed publisher
western blot; human; 1:1000; loading ...; fig 6a	Zha J, Liu X, Zhu J, Liu S, Lu S, Xu P, et al. A scFv antibody targeting common oligomeric epitope has potential for treating several amyloidoses. Sci Rep. 2016;6:36631 pubmed publisher
western blot; mouse; 1:1000; loading ...; fig 2c	Rue L, Bañez Coronel M, Creus Muncunill J, Giralt A, Alcalá Vida R, Mentxaka G, et al. Targeting CAG repeat RNAs reduces Huntington's disease phenotype independently of huntingtin levels. J Clin Invest. 2016;126:4319-4330 pubmed publisher
western blot; mouse; 1:1000; fig 1c	Guo X, Sun X, Hu D, Wang Y, Fujioka H, Vyas R, et al. VCP recruitment to mitochondria causes mitophagy impairment and neurodegeneration in models of Huntington's disease. Nat Commun. 2016;7:12646 pubmed publisher
western blot; mouse; 1:3000; loading ...; fig s3a	Kratter I, Zahed H, Lau A, Tsvetkov A, Daub A, Weiberth K, et al. Serine 421 regulates mutant huntingtin toxicity and clearance in mice. J Clin Invest. 2016;126:3585-97 pubmed publisher
western blot; mouse; 1:1000; loading ...; fig 6a	Vodicka P, Chase K, Iuliano M, Valentine D, Sapp E, Lu B, et al. Effects of Exogenous NUB1 Expression in the Striatum of HDQ175/Q7 Mice. J Huntingtons Dis. 2016;5:163-74 pubmed publisher
immunohistochemistry - paraffin section; mouse; 1:500; fig s2	Lewis M, Buniello A, Hilton J, Zhu F, Zhang W, Evans S, et al. Exploring regulatory networks of miR-96 in the developing inner ear. Sci Rep. 2016;6:23363 pubmed publisher
western blot; mouse; 1:1000; loading ...; fig 1a	Culver B, DeClercq J, Dolgalev I, Yu M, Ma B, Heguy A, et al. Huntington's Disease Protein Huntingtin Associates with its own mRNA. J Huntingtons Dis. 2016;5:39-51 pubmed publisher
western blot; rat; fig s7	Wang J, Cao Y, Li Q, Yang Y, Jin M, Chen D, et al. A pivotal role of FOS-mediated BECN1/Beclin 1 upregulation in dopamine D2 and D3 receptor agonist-induced autophagy activation. Autophagy. 2015;11:2057-2073 pubmed publisher
western blot; mouse; fig 4	Hwang S, Disatnik M, Mochly Rosen D. Impaired GAPDH-induced mitophagy contributes to the pathology of Huntington's disease. EMBO Mol Med. 2015;7:1307-26 pubmed publisher
flow cytometry; mouse western blot; mouse; 1:2000; loading ...; fig 3b	Southwell A, Smith S, Davis T, Caron N, Villanueva E, Xie Y, et al. Ultrasensitive measurement of huntingtin protein in cerebrospinal fluid demonstrates increase with Huntington disease stage and decrease following brain huntingtin suppression. Sci Rep. 2015;5:12166 pubmed publisher
western blot; mouse; 1:200; fig 2	Szlachcic W, Switonski P, Krzyzosiak W, Figlerowicz M, Figiel M. Huntington disease iPSCs show early molecular changes in intracellular signaling, the expression of oxidative stress proteins and the p53 pathway. Dis Model Mech. 2015;8:1047-57 pubmed publisher
immunoprecipitation; mouse western blot; mouse; 1:1000 immunoprecipitation; human western blot; human; 1:1000	O Brien R, DeGiacomo F, Holcomb J, Bonner A, Ring K, Zhang N, et al. Integration-independent Transgenic Huntington Disease Fragment Mouse Models Reveal Distinct Phenotypes and Life Span in Vivo. J Biol Chem. 2015;290:19287-306 pubmed publisher
western blot; mouse; fig 4	Cheng H, Chern Y, Chen I, Liu C, Li S, Chun S, et al. Effects on murine behavior and lifespan of selectively decreasing expression of mutant huntingtin allele by supt4h knockdown. PLoS Genet. 2015;11:e1005043 pubmed publisher
western blot; mouse; 1:1000; fig 1	Yao Y, Cui X, Al Ramahi I, Sun X, Li B, Hou J, et al. A striatal-enriched intronic GPCR modulates huntingtin levels and toxicity. elife. 2015;4: pubmed publisher
western blot; mouse	Sanders S, Hou J, Sutton L, Garside V, Mui K, Singaraja R, et al. Huntingtin interacting proteins 14 and 14-like are required for chorioallantoic fusion during early placental development. Dev Biol. 2015;397:257-66 pubmed publisher
western blot; human	Brandstaetter H, Kruppa A, Buss F. Huntingtin is required for ER-to-Golgi transport and for secretory vesicle fusion at the plasma membrane. Dis Model Mech. 2014;7:1335-40 pubmed publisher
western blot; mouse; 1:2000	Graham R, Deng Y, Pouladi M, Vaid K, Ehrnhoefer D, Southwell A, et al. Caspase-6-Resistant Mutant Huntingtin Does not Rescue the Toxic Effects of Caspase-Cleavable Mutant Huntingtin in vivo. J Huntingtons Dis. 2012;1:243-60 pubmed publisher
immunohistochemistry - paraffin section; human; 1:150	Simeone P, Trerotola M, Urbanella A, Lattanzio R, Ciavardelli D, Di Giuseppe F, et al. A unique four-hub protein cluster associates to glioblastoma progression. PLoS ONE. 2014;9:e103030 pubmed publisher
western blot; human; 1:10000	Macdonald D, Tessari M, Boogaard I, Smith M, Pulli K, Szynol A, et al. Quantification assays for total and polyglutamine-expanded huntingtin proteins. PLoS ONE. 2014;9:e96854 pubmed publisher
immunohistochemistry - paraffin section; human; 1:500	Mori F, Watanabe Y, Miki Y, Tanji K, Odagiri S, Eto K, et al. Ubiquitin-negative, eosinophilic neuronal cytoplasmic inclusions associated with stress granules and autophagy: an immunohistochemical investigation of two cases. Neuropathology. 2014;34:140-7 pubmed
western blot; human; 1:1000; fig 6	Cicchetti F, Lacroix S, Cisbani G, Vallières N, Saint Pierre M, St Amour I, et al. Mutant huntingtin is present in neuronal grafts in Huntington disease patients. Ann Neurol. 2014;76:31-42 pubmed publisher
western blot; human; 1:1000	An M, O Brien R, Zhang N, Patra B, de la Cruz M, Ray A, et al. Polyglutamine Disease Modeling: Epitope Based Screen for Homologous Recombination using CRISPR/Cas9 System. PLoS Curr. 2014;6: pubmed publisher
western blot; African green monkey; 1:1000	Sanders S, Mui K, Sutton L, Hayden M. Identification of binding sites in Huntingtin for the Huntingtin Interacting Proteins HIP14 and HIP14L. PLoS ONE. 2014;9:e90669 pubmed publisher
immunohistochemistry - paraffin section; human; 1:200	Schiffer D, Caldera V, Mellai M, Conforti P, Cattaneo E, Zuccato C. Repressor element-1 silencing transcription factor (REST) is present in human control and Huntington's disease neurones. Neuropathol Appl Neurobiol. 2014;40:899-910 pubmed publisher
immunoprecipitation; human western blot; human	Marion S, Urs N, Peterson S, Sotnikova T, Beaulieu J, Gainetdinov R, et al. Dopamine D2 receptor relies upon PPM/PP2C protein phosphatases to dephosphorylate huntingtin protein. J Biol Chem. 2014;289:11715-24 pubmed publisher
western blot; human	Fernandez Estevez M, Casarejos M, Lopez Sendon J, Garcia Caldentey J, Ruiz C, Gomez A, et al. Trehalose reverses cell malfunction in fibroblasts from normal and Huntington's disease patients caused by proteosome inhibition. PLoS ONE. 2014;9:e90202 pubmed publisher
western blot; mouse	Wong Y, Holzbaur E. The regulation of autophagosome dynamics by huntingtin and HAP1 is disrupted by expression of mutant huntingtin, leading to defective cargo degradation. J Neurosci. 2014;34:1293-305 pubmed publisher
western blot; human; 1:1,000	Evers M, Tran H, Zalachoras I, Meijer O, den Dunnen J, van Ommen G, et al. Preventing formation of toxic N-terminal huntingtin fragments through antisense oligonucleotide-mediated protein modification. Nucleic Acid Ther. 2014;24:4-12 pubmed publisher
western blot; mouse; 1:2000	Baldo B, Soylu R, Petersen A. Maintenance of basal levels of autophagy in Huntington's disease mouse models displaying metabolic dysfunction. PLoS ONE. 2013;8:e83050 pubmed publisher
western blot; human	Aiba Y, Hu J, Liu J, Xiang Q, Martinez C, Corey D. Allele-selective inhibition of expression of huntingtin and ataxin-3 by RNA duplexes containing unlocked nucleic acid substitutions. Biochemistry. 2013;52:9329-38 pubmed publisher
immunoprecipitation; human	Sbodio J, Paul B, Machamer C, Snyder S. Golgi protein ACBD3 mediates neurotoxicity associated with Huntington's disease. Cell Rep. 2013;4:890-7 pubmed publisher
western blot; human	Liu J, Pendergraff H, Narayanannair K, Lackey J, Kuchimanchi S, Rajeev K, et al. RNA duplexes with abasic substitutions are potent and allele-selective inhibitors of huntingtin and ataxin-3 expression. Nucleic Acids Res. 2013;41:8788-801 pubmed publisher
western blot; mouse	Strong M, Southwell A, Yonan J, Hayden M, MacGregor G, Thompson L, et al. Age-Dependent Resistance to Excitotoxicity in Htt CAG140 Mice and the Effect of Strain Background. J Huntingtons Dis. 2012;1:221-41 pubmed publisher
immunohistochemistry - paraffin section; mouse; 1:50	Milman P, Woulfe J. Novel variant of neuronal intranuclear rodlet immunoreactive for 40 kDa huntingtin associated protein and ubiquitin in the mouse brain. J Comp Neurol. 2013;521:3832-46 pubmed publisher
western blot; mouse; 1:250	Silva A, Almeida S, Laço M, Duarte A, Domingues J, Oliveira C, et al. Mitochondrial respiratory chain complex activity and bioenergetic alterations in human platelets derived from pre-symptomatic and symptomatic Huntington's disease carriers. Mitochondrion. 2013;13:801-9 pubmed publisher
immunohistochemistry; rat western blot; rat	Haun F, Nakamura T, Shiu A, Cho D, Tsunemi T, Holland E, et al. S-nitrosylation of dynamin-related protein 1 mediates mutant huntingtin-induced mitochondrial fragmentation and neuronal injury in Huntington's disease. Antioxid Redox Signal. 2013;19:1173-84 pubmed publisher
western blot; rat; 1:2,500	Godinho B, Ogier J, Darcy R, O Driscoll C, Cryan J. Self-assembling modified ?-cyclodextrin nanoparticles as neuronal siRNA delivery vectors: focus on Huntington's disease. Mol Pharm. 2013;10:640-9 pubmed publisher
western blot; human; 1:2000 western blot; rat; 1:2000	Yu Taeger L, Petrasch Parwez E, Osmand A, Redensek A, Metzger S, Clemens L, et al. A novel BACHD transgenic rat exhibits characteristic neuropathological features of Huntington disease. J Neurosci. 2012;32:15426-38 pubmed publisher
immunoprecipitation; human immunocytochemistry; human western blot; human; fig 5c	Qi L, Zhang X, Wu J, Lin F, Wang J, DiFiglia M, et al. The role of chaperone-mediated autophagy in huntingtin degradation. PLoS ONE. 2012;7:e46834 pubmed publisher
western blot; human	Hu J, Liu J, Yu D, Chu Y, Corey D. Mechanism of allele-selective inhibition of huntingtin expression by duplex RNAs that target CAG repeats: function through the RNAi pathway. Nucleic Acids Res. 2012;40:11270-80 pubmed publisher
	Sharma M, Subramaniam S. Rhes travels from cell to cell and transports Huntington disease protein via TNT-like protrusion. J Cell Biol. 2019;218:1972-1993 pubmed publisher
	Ooi J, Langley S, Xu X, Utami K, Sim B, Huang Y, et al. Unbiased Profiling of Isogenic Huntington Disease hPSC-Derived CNS and Peripheral Cells Reveals Strong Cell-Type Specificity of CAG Length Effects. Cell Rep. 2019;26:2494-2508.e7 pubmed publisher
	Masnata M, Sciacca G, Maxan A, Bousset L, Denis H, Lauruol F, et al. Demonstration of prion-like properties of mutant huntingtin fibrils in both in vitro and in vivo paradigms. Acta Neuropathol. 2019;137:981-1001 pubmed publisher
	Tousley A, Iuliano M, Weisman E, Sapp E, Richardson H, Vodicka P, et al. Huntingtin associates with the actin cytoskeleton and α-actinin isoforms to influence stimulus dependent morphology changes. PLoS ONE. 2019;14:e0212337 pubmed publisher
	Tousley A, Iuliano M, Weisman E, Sapp E, Zhang N, Vodicka P, et al. Rac1 Activity Is Modulated by Huntingtin and Dysregulated in Models of Huntington's Disease. J Huntingtons Dis. 2019;8:53-69 pubmed publisher
	Hung C, Maiuri T, Bowie L, Gotesman R, Son S, Falcone M, et al. A patient-derived cellular model for Huntington's disease reveals phenotypes at clinically relevant CAG lengths. Mol Biol Cell. 2018;29:2809-2820 pubmed publisher
	Mueller K, Glajch K, Huizenga M, Wilson R, Granucci E, Dios A, et al. Hippo Signaling Pathway Dysregulation in Human Huntington's Disease Brain and Neuronal Stem Cells. Sci Rep. 2018;8:11355 pubmed publisher
	Franich N, Basso M, ANDRE E, Ochaba J, Kumar A, Thein S, et al. Striatal Mutant Huntingtin Protein Levels Decline with Age in Homozygous Huntington's Disease Knock-In Mouse Models. J Huntingtons Dis. 2018;7:137-150 pubmed publisher
	Dabrowska M, Juzwa W, Krzyzosiak W, Olejniczak M. Precise Excision of the CAG Tract from the Huntingtin Gene by Cas9 Nickases. Front Neurosci. 2018;12:75 pubmed publisher
	Guo Q, Bin Huang -, Cheng J, Seefelder M, Engler T, Pfeifer G, et al. The cryo-electron microscopy structure of huntingtin. Nature. 2018;555:117-120 pubmed publisher
	Al Ramahi I, Giridharan S, Chen Y, Patnaik S, Safren N, Hasegawa J, et al. Inhibition of PIP4K? ameliorates the pathological effects of mutant huntingtin protein. elife. 2017;6: pubmed publisher
	Ma Q, Yang J, Milner T, Vonsattel J, Palko M, Tessarollo L, et al. SorCS2-mediated NR2A trafficking regulates motor deficits in Huntington's disease. JCI Insight. 2017;2: pubmed publisher
	Urbanek M, Fiszer A, Krzyzosiak W. Reduction of Huntington's Disease RNA Foci by CAG Repeat-Targeting Reagents. Front Cell Neurosci. 2017;11:82 pubmed publisher
	Shin J, Kim K, Chao M, Atwal R, Gillis T, MacDonald M, et al. Permanent inactivation of Huntington's disease mutation by personalized allele-specific CRISPR/Cas9. Hum Mol Genet. 2016;25:4566-4576 pubmed publisher
	Miniarikova J, Zanella I, Huseinovic A, van der Zon T, Hanemaaijer E, Martier R, et al. Design, Characterization, and Lead Selection of Therapeutic miRNAs Targeting Huntingtin for Development of Gene Therapy for Huntington's Disease. Mol Ther Nucleic Acids. 2016;5:e297 pubmed publisher
	Vijayvargia R, Epand R, Leitner A, Jung T, Shin B, Jung R, et al. Huntingtin's spherical solenoid structure enables polyglutamine tract-dependent modulation of its structure and function. elife. 2016;5:e11184 pubmed publisher
	Bowles K, Brooks S, Dunnett S, Jones L. Huntingtin Subcellular Localisation Is Regulated by Kinase Signalling Activity in the StHdhQ111 Model of HD. PLoS ONE. 2015;10:e0144864 pubmed publisher
	Dickey A, Pineda V, Tsunemi T, Liu P, Miranda H, Gilmore Hall S, et al. PPAR-Î´ is repressed in Huntington's disease, is required for normal neuronal function and can be targeted therapeutically. Nat Med. 2016;22:37-45 pubmed publisher
	Sun X, Li P, Zhu S, Cohen R, Marque L, Ross C, et al. Nuclear retention of full-length HTT RNA is mediated by splicing factors MBNL1 and U2AF65. Sci Rep. 2015;5:12521 pubmed publisher
	Huang B, Lucas T, Kueppers C, Dong X, Krause M, Bepperling A, et al. Scalable production in human cells and biochemical characterization of full-length normal and mutant huntingtin. PLoS ONE. 2015;10:e0121055 pubmed publisher
	Fodale V, Kegulian N, Verani M, Cariulo C, Azzollini L, Petricca L, et al. Polyglutamine- and temperature-dependent conformational rigidity in mutant huntingtin revealed by immunoassays and circular dichroism spectroscopy. PLoS ONE. 2014;9:e112262 pubmed publisher
	Southwell A, Skotte N, Kordasiewicz H, Østergaard M, Watt A, Carroll J, et al. In vivo evaluation of candidate allele-specific mutant huntingtin gene silencing antisense oligonucleotides. Mol Ther. 2014;22:2093-106 pubmed publisher
	Stanek L, Yang W, Angus S, Sardi P, Hayden M, Hung G, et al. Antisense oligonucleotide-mediated correction of transcriptional dysregulation is correlated with behavioral benefits in the YAC128 mouse model of Huntington's disease. J Huntingtons Dis. 2013;2:217-28 pubmed publisher
	Marques Sousa C, Humbert S. Huntingtin: here, there, everywhere!. J Huntingtons Dis. 2013;2:395-403 pubmed publisher
	Reid S, Patassini S, Handley R, Rudiger S, McLaughlan C, Osmand A, et al. Further molecular characterisation of the OVT73 transgenic sheep model of Huntington's disease identifies cortical aggregates. J Huntingtons Dis. 2013;2:279-95 pubmed publisher
	Stanek L, Sardi S, Mastis B, Richards A, Treleaven C, Taksir T, et al. Silencing mutant huntingtin by adeno-associated virus-mediated RNA interference ameliorates disease manifestations in the YAC128 mouse model of Huntington's disease. Hum Gene Ther. 2014;25:461-74 pubmed publisher
	Acuña A, Esparza M, Kramm C, Beltrán F, Parra A, Cepeda C, et al. A failure in energy metabolism and antioxidant uptake precede symptoms of Huntington's disease in mice. Nat Commun. 2013;4:2917 pubmed publisher
	Quintanilla R, Jin Y, von Bernhardi R, Johnson G. Mitochondrial permeability transition pore induces mitochondria injury in Huntington disease. Mol Neurodegener. 2013;8:45 pubmed publisher
	Prévilon M, Le Gall M, Chafey P, Federeci C, Pezet M, Clary G, et al. Comparative differential proteomic profiles of nonfailing and failing hearts after in vivo thoracic aortic constriction in mice overexpressing FKBP12.6. Physiol Rep. 2013;1:e00039 pubmed publisher
	Sontag E, Lotz G, Yang G, Sontag C, Cummings B, Glabe C, et al. Detection of Mutant Huntingtin Aggregation Conformers and Modulation of SDS-Soluble Fibrillar Oligomers by Small Molecules. J Huntingtons Dis. 2012;1:119-32 pubmed publisher
	Pla P, Orvoen S, Benstaali C, Dodier S, Gardier A, David D, et al. Huntingtin acts non cell-autonomously on hippocampal neurogenesis and controls anxiety-related behaviors in adult mouse. PLoS ONE. 2013;8:e73902 pubmed publisher
	Marco S, Giralt A, Petrovic M, Pouladi M, Mart nez Turrillas R, Mart nez Hern ndez J, et al. Suppressing aberrant GluN3A expression rescues synaptic and behavioral impairments in Huntington's disease models. Nat Med. 2013;19:1030-8 pubmed publisher
	Cheng P, Li C, Chang Y, Tsai S, Lai Y, Chan A, et al. miR-196a ameliorates phenotypes of Huntington disease in cell, transgenic mouse, and induced pluripotent stem cell models. Am J Hum Genet. 2013;93:306-12 pubmed publisher
	Niclis J, Pinar A, Haynes J, Alsanie W, Jenny R, Dottori M, et al. Characterization of forebrain neurons derived from late-onset Huntington's disease human embryonic stem cell lines. Front Cell Neurosci. 2013;7:37 pubmed publisher
	Lu B, Palacino J. A novel human embryonic stem cell-derived Huntington's disease neuronal model exhibits mutant huntingtin (mHTT) aggregates and soluble mHTT-dependent neurodegeneration. FASEB J. 2013;27:1820-9 pubmed publisher
	Rue L, López Soop G, Gelpi E, Martinez Vicente M, Alberch J, Pérez Navarro E. Brain region- and age-dependent dysregulation of p62 and NBR1 in a mouse model of Huntington's disease. Neurobiol Dis. 2013;52:219-28 pubmed publisher
	Miller J, Yates B, Al Ramahi I, Berman A, Sanhueza M, Kim E, et al. A genome-scale RNA-interference screen identifies RRAS signaling as a pathologic feature of Huntington's disease. PLoS Genet. 2012;8:e1003042 pubmed publisher
	Garriga Canut M, Agustín Pavón C, Herrmann F, Sanchez A, Dierssen M, Fillat C, et al. Synthetic zinc finger repressors reduce mutant huntingtin expression in the brain of R6/2 mice. Proc Natl Acad Sci U S A. 2012;109:E3136-45 pubmed publisher
	Neveklovska M, Clabough E, Steffan J, Zeitlin S. Deletion of the huntingtin proline-rich region does not significantly affect normal huntingtin function in mice. J Huntingtons Dis. 2012;1:71-87 pubmed publisher
	Zheng S, Ghitani N, Blackburn J, Liu J, Zeitlin S. A series of N-terminal epitope tagged Hdh knock-in alleles expressing normal and mutant huntingtin: their application to understanding the effect of increasing the length of normal Huntingtin's polyglutamine stretch on CAG140 mouse model pathogenesis. Mol Brain. 2012;5:28 pubmed publisher
	Hogel M, Laprairie R, Denovan Wright E. Promoters are differentially sensitive to N-terminal mutant huntingtin-mediated transcriptional repression. PLoS ONE. 2012;7:e41152 pubmed publisher
	Dong X, Zong S, Witting A, Lindenberg K, Kochanek S, Huang B. Adenovirus vector-based in vitro neuronal cell model for Huntington's disease with human disease-like differential aggregation and degeneration. J Gene Med. 2012;14:468-81 pubmed publisher
	Juopperi T, Kim W, Chiang C, Yu H, Margolis R, Ross C, et al. Astrocytes generated from patient induced pluripotent stem cells recapitulate features of Huntington's disease patient cells. Mol Brain. 2012;5:17 pubmed publisher
	Culver B, Savas J, Park S, Choi J, Zheng S, Zeitlin S, et al. Proteomic analysis of wild-type and mutant huntingtin-associated proteins in mouse brains identifies unique interactions and involvement in protein synthesis. J Biol Chem. 2012;287:21599-614 pubmed publisher
	Yuen E, Wei J, Zhong P, Yan Z. Disrupted GABAAR trafficking and synaptic inhibition in a mouse model of Huntington's disease. Neurobiol Dis. 2012;46:497-502 pubmed publisher
	Fiszer A, Olejniczak M, Switonski P, Wroblewska J, Wisniewska Kruk J, Mykowska A, et al. An evaluation of oligonucleotide-based therapeutic strategies for polyQ diseases. BMC Mol Biol. 2012;13:6 pubmed publisher
	Sapp E, Valencia A, Li X, Aronin N, Kegel K, Vonsattel J, et al. Native mutant huntingtin in human brain: evidence for prevalence of full-length monomer. J Biol Chem. 2012;287:13487-99 pubmed publisher
	Ma B, Savas J, Yu M, Culver B, Chao M, Tanese N. Huntingtin mediates dendritic transport of ?-actin mRNA in rat neurons. Sci Rep. 2011;1:140 pubmed publisher
	Chow W, Luk H, Chan H, Lau K. Degradation of mutant huntingtin via the ubiquitin/proteasome system is modulated by FE65. Biochem J. 2012;443:681-9 pubmed publisher
	Sedaghat Y, Mazur C, Sabripour M, Hung G, Monia B. Genomic analysis of wig-1 pathways. PLoS ONE. 2012;7:e29429 pubmed publisher
	Chen L, Wu J, Wang L, Wang J, Qin Z, DiFiglia M, et al. Rapamycin prevents the mutant huntingtin-suppressed GLT-1 expression in cultured astrocytes. Acta Pharmacol Sin. 2012;33:385-92 pubmed publisher
	Grondin R, Kaytor M, Ai Y, Nelson P, Thakker D, Heisel J, et al. Six-month partial suppression of Huntingtin is well tolerated in the adult rhesus striatum. Brain. 2012;135:1197-209 pubmed publisher
	Jeong H, Cohen D, Cui L, Supinski A, Savas J, Mazzulli J, et al. Sirt1 mediates neuroprotection from mutant huntingtin by activation of the TORC1 and CREB transcriptional pathway. Nat Med. 2011;18:159-65 pubmed publisher
	Stiles D, Zhang Z, Ge P, Nelson B, Grondin R, Ai Y, et al. Widespread suppression of huntingtin with convection-enhanced delivery of siRNA. Exp Neurol. 2012;233:463-71 pubmed publisher
	Weiss K, Kimura Y, Lee W, Littleton J. Huntingtin aggregation kinetics and their pathological role in a Drosophila Huntington's disease model. Genetics. 2012;190:581-600 pubmed publisher
	Tong Y, Ha T, Liu L, Nishimoto A, Reiner A, Goldowitz D. Spatial and temporal requirements for huntingtin (Htt) in neuronal migration and survival during brain development. J Neurosci. 2011;31:14794-9 pubmed publisher
	Carroll J, Warby S, Southwell A, Doty C, Greenlee S, Skotte N, et al. Potent and selective antisense oligonucleotides targeting single-nucleotide polymorphisms in the Huntington disease gene / allele-specific silencing of mutant huntingtin. Mol Ther. 2011;19:2178-85 pubmed publisher
	Schulte J, Sepp K, Wu C, Hong P, Littleton J. High-content chemical and RNAi screens for suppressors of neurotoxicity in a Huntington's disease model. PLoS ONE. 2011;6:e23841 pubmed publisher
	Zucchelli S, Marcuzzi F, Codrich M, Agostoni E, Vilotti S, Biagioli M, et al. Tumor necrosis factor receptor-associated factor 6 (TRAF6) associates with huntingtin protein and promotes its atypical ubiquitination to enhance aggregate formation. J Biol Chem. 2011;286:25108-17 pubmed publisher
	Reis S, Thompson M, Lee J, Fossale E, Kim H, Liao J, et al. Striatal neurons expressing full-length mutant huntingtin exhibit decreased N-cadherin and altered neuritogenesis. Hum Mol Genet. 2011;20:2344-55 pubmed publisher
	Cai L, Bian M, Liu M, Sheng Z, Suo H, Wang Z, et al. Ethanol-induced neurodegeneration in NRSF/REST neuronal conditional knockout mice. Neuroscience. 2011;181:196-205 pubmed publisher
	Sassone J, Colciago C, Marchi P, Ascardi C, Alberti L, Di Pardo A, et al. Mutant Huntingtin induces activation of the Bcl-2/adenovirus E1B 19-kDa interacting protein (BNip3). Cell Death Dis. 2010;1:e7 pubmed publisher
	Munsie L, Caron N, Atwal R, Marsden I, Wild E, Bamburg J, et al. Mutant huntingtin causes defective actin remodeling during stress: defining a new role for transglutaminase 2 in neurodegenerative disease. Hum Mol Genet. 2011;20:1937-51 pubmed publisher
	Ellrichmann G, Petrasch Parwez E, Lee D, Reick C, Arning L, Saft C, et al. Efficacy of fumaric acid esters in the R6/2 and YAC128 models of Huntington's disease. PLoS ONE. 2011;6:e16172 pubmed publisher
	Moscovitch Lopatin M, Weiss A, ROSAS H, Ritch J, Doros G, Kegel K, et al. Optimization of an HTRF Assay for the Detection of Soluble Mutant Huntingtin in Human Buffy Coats: A Potential Biomarker in Blood for Huntington Disease. PLoS Curr. 2010;2:RRN1205 pubmed publisher
	Poksay K, Madden D, Peter A, Niazi K, Banwait S, Crippen D, et al. Valosin-containing protein gene mutations: cellular phenotypes relevant to neurodegeneration. J Mol Neurosci. 2011;44:91-102 pubmed publisher
	Gagnon K, Pendergraff H, Deleavey G, Swayze E, Potier P, Randolph J, et al. Allele-selective inhibition of mutant huntingtin expression with antisense oligonucleotides targeting the expanded CAG repeat. Biochemistry. 2010;49:10166-78 pubmed publisher
	Varma H, Yamamoto A, Sarantos M, Hughes R, Stockwell B. Mutant huntingtin alters cell fate in response to microtubule depolymerization via the GEF-H1-RhoA-ERK pathway. J Biol Chem. 2010;285:37445-57 pubmed publisher
	Sheeba V, Fogle K, Holmes T. Persistence of morning anticipation behavior and high amplitude morning startle response following functional loss of small ventral lateral neurons in Drosophila. PLoS ONE. 2010;5:e11628 pubmed publisher
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product information

Catalog Number :

MAB2166

Subcategory :

Neuroscience

Product Name :

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

Product Type :

Antibodies

Clonality :

Monoclonal Antibody

Gene ID :

P42858

Host Name :

Mouse

Antigen :

Huntingtin Protein

Clone :

1HU-4C8

Conjugate :

Ascites

Isotype :

IgG1κ

Product Description :

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

Cross Reactivity :

Human;Mouse;Rat;Monkey;Hamster;Rabbit

Background :

Huntington 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.

ALT Names :

Huntingtin;Huntington's Disease Protein;HD Protein

Immunogen :

Huntingtin fragment from a.a. 181 to 810 as a fusion protein.

Specificity :

Huntingtin Protein. No detectable cross reactivity to other proteins by Western blot.

Package Size :

100 µL

Uses :

ELISA;Immunocytochemistry;Immunohistochemistry (Paraffin);Immunoprecipitation;Western Blotting

Storage :

Stable 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.