产品简要
公司名称 :
默克密理博中国
其他品牌 :
Oncogene Research Products, Calbiochem, Novagen, Merck, Upstate Biotechnology, Chemicon, LINCO, Novabiochem, Guava
产品类型 :
抗体
产品名称 :
Huntingtin Protein Antibody, clone mEM48
目录 :
MAB5374
规格 :
100微升
克隆性 :
单克隆
宿主 :
小鼠
共轭标签 :
未共轭
克隆名称 :
mEM48
反应物种 :
人类, 小鼠, 大鼠, S. cerevisiae
应用 :
免疫印迹, 免疫组化, 免疫细胞化学, 免疫组化-石蜡切片, 免疫组化-冰冻切片, 免疫组化-自由浮动切片
更多信息或购买 :
文章摘录数: 103
出版应用/物种/样本/稀释参考文献
  • 免疫组化-自由浮动切片; 小鼠; 1:100; 图 4d
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 出版商
  • 免疫组化-冰冻切片; 小鼠; 1:300; 图 7a
Fukuoka M, Takahashi M, Fujita H, Chiyo T, Popiel H, Watanabe S, et al. Supplemental Treatment for Huntington's Disease with miR-132 that Is Deficient in Huntington's Disease Brain. Mol Ther Nucleic Acids. 2018;11:79-90 pubmed 出版商
  • 免疫细胞化学; 人类; 1:50; 图 4c
Victor M, Richner M, Olsen H, Lee S, Monteys A, Ma C, et al. Striatal neurons directly converted from Huntington's disease patient fibroblasts recapitulate age-associated disease phenotypes. Nat Neurosci. 2018;21:341-352 pubmed 出版商
  • 免疫印迹; 人类; 1:500; 图 6
Kolli N, Lu M, Maiti P, Rossignol J, Dunbar G. CRISPR-Cas9 Mediated Gene-Silencing of the Mutant Huntingtin Gene in an In Vitro Model of Huntington's Disease. Int J Mol Sci. 2017;18: pubmed 出版商
  • 免疫印迹; 小鼠; 1:500; 图 8
Huang Z, Her L. The Ubiquitin Receptor ADRM1 Modulates HAP40-Induced Proteasome Activity. Mol Neurobiol. 2017;54:7382-7400 pubmed 出版商
  • 免疫印迹; S. cerevisiae; 图 1c
  • 免疫印迹; 人类; 1:1000; 图 2f
Vicente Miranda H, Gomes M, Branco Santos J, Breda C, Lázaro D, Lopes L, et al. Glycation potentiates neurodegeneration in models of Huntington's disease. Sci Rep. 2016;6:36798 pubmed 出版商
  • 免疫组化-冰冻切片; 小鼠; 1:50; 图 7a
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 出版商
  • 免疫印迹; 小鼠; 1:1000; 图 2b
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 出版商
  • 免疫组化; 小鼠; 1:100; 图 1
Agostoni E, Michelazzi S, Maurutto M, Carnemolla A, Ciani Y, Vatta P, et al. Effects of Pin1 Loss in Hdh(Q111) Knock-in Mice. Front Cell Neurosci. 2016;10:110 pubmed 出版商
  • 免疫组化-冰冻切片; 小鼠; 1:500; 图 2
Bayram Weston Z, Jones L, Dunnett S, Brooks S. Comparison of mHTT Antibodies in Huntington's Disease Mouse Models Reveal Specific Binding Profiles and Steady-State Ubiquitin Levels with Disease Development. PLoS ONE. 2016;11:e0155834 pubmed 出版商
  • 免疫印迹; 小鼠; 1:1000; 图 2
Peng Y, Kim M, Hullinger R, O Riordan K, Burger C, Pehar M, et al. Improved proteostasis in the secretory pathway rescues Alzheimer's disease in the mouse. Brain. 2016;139:937-52 pubmed 出版商
  • 免疫组化-石蜡切片; 小鼠; 1:50
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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 出版商
  • 免疫组化-冰冻切片; 小鼠; 图 6e
Zhang T, Dong K, Liang W, Xu D, Xia H, Geng J, et al. G-protein-coupled receptors regulate autophagy by ZBTB16-mediated ubiquitination and proteasomal degradation of Atg14L. elife. 2015;4:e06734 pubmed 出版商
  • 免疫组化-石蜡切片; 小鼠
  • 免疫细胞化学; 小鼠; 图 1
Shen W, Li H, Chen G, Chern Y, Tu P. Mutations in the ubiquitin-binding domain of OPTN/optineurin interfere with autophagy-mediated degradation of misfolded proteins by a dominant-negative mechanism. Autophagy. 2015;11:685-700 pubmed 出版商
  • 免疫组化-自由浮动切片; 小鼠; 1:100
Doria J, de Souza J, Andrade J, Rodrigues H, Guimaraes I, Carvalho T, et al. The mGluR5 positive allosteric modulator, CDPPB, ameliorates pathology and phenotypic signs of a mouse model of Huntington's disease. Neurobiol Dis. 2015;73:163-73 pubmed 出版商
  • 免疫组化-石蜡切片; 小鼠; 1:500
Blum D, Herrera F, Francelle L, Mendes T, Basquin M, Obriot H, et al. Mutant huntingtin alters Tau phosphorylation and subcellular distribution. Hum Mol Genet. 2015;24:76-85 pubmed 出版商
  • 免疫组化-自由浮动切片; 人类
Corrochano S, Renna M, Osborne G, Carter S, Stewart M, May J, et al. Reducing Igf-1r levels leads to paradoxical and sexually dimorphic effects in HD mice. PLoS ONE. 2014;9:e105595 pubmed 出版商
  • 免疫细胞化学; 大鼠; 1:1000
  • 免疫印迹; 大鼠; 1:1000
Stansfield K, Bichell T, Bowman A, Guilarte T. BDNF and Huntingtin protein modifications by manganese: implications for striatal medium spiny neuron pathology in manganese neurotoxicity. J Neurochem. 2014;131:655-66 pubmed 出版商
  • 免疫印迹; 小鼠; 1:1000
Zhang H, Petit G, Gaughwin P, Hansen C, Ranganathan S, Zuo X, et al. NGF rescues hippocampal cholinergic neuronal markers, restores neurogenesis, and improves the spatial working memory in a mouse model of Huntington's Disease. J Huntingtons Dis. 2013;2:69-82 pubmed 出版商
  • 免疫组化-石蜡切片; 人类; 1:100
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Fernández Nogales M, Cabrera J, Santos Galindo M, Hoozemans J, Ferrer I, Rozemuller A, et al. Huntington's disease is a four-repeat tauopathy with tau nuclear rods. Nat Med. 2014;20:881-5 pubmed 出版商
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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 出版商
  • 免疫印迹; 大鼠; 1:1000
Lai A, Lan C, Hasan S, Brown M, McLaurin J. scyllo-Inositol promotes robust mutant Huntingtin protein degradation. J Biol Chem. 2014;289:3666-76 pubmed 出版商
  • 免疫印迹; 小鼠; 1:100
Perucho J, Casarejos M, Gomez A, Ruiz C, Fernandez Estevez M, Muñoz M, et al. Striatal infusion of glial conditioned medium diminishes huntingtin pathology in r6/1 mice. PLoS ONE. 2013;8:e73120 pubmed 出版商
  • 免疫组化; 小鼠
Valor L, Guiretti D, Lopez Atalaya J, Barco A. Genomic landscape of transcriptional and epigenetic dysregulation in early onset polyglutamine disease. J Neurosci. 2013;33:10471-82 pubmed 出版商
  • 免疫细胞化学; 人类
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 出版商
  • 免疫组化-冰冻切片; 小鼠; 1:250
Popiel H, Takeuchi T, Fujita H, Yamamoto K, Ito C, Yamane H, et al. Hsp40 gene therapy exerts therapeutic effects on polyglutamine disease mice via a non-cell autonomous mechanism. PLoS ONE. 2012;7:e51069 pubmed 出版商
  • 免疫组化-自由浮动切片; 小鼠; 1:300
Horne E, Coy J, Swinney K, Fung S, Cherry A, Marrs W, et al. Downregulation of cannabinoid receptor 1 from neuropeptide Y interneurons in the basal ganglia of patients with Huntington's disease and mouse models. Eur J Neurosci. 2013;37:429-40 pubmed 出版商
  • 免疫印迹; 小鼠; 图 2
Rahman A, Ekman M, Shakirova Y, Andersson K, Morgelin M, Erjefalt J, et al. Late onset vascular dysfunction in the R6/1 model of Huntington's disease. Eur J Pharmacol. 2013;698:345-53 pubmed 出版商
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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 出版商
Ekman F, Ojala D, Adil M, Lopez P, Schaffer D, Gaj T. CRISPR-Cas9-Mediated Genome Editing Increases Lifespan and Improves Motor Deficits in a Huntington's Disease Mouse Model. Mol Ther Nucleic Acids. 2019;17:829-839 pubmed 出版商
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Schwab C, Yu S, McGeer P. Optineurin is colocalized with ubiquitin in Marinesco bodies. Acta Neuropathol. 2012;123:289-92 pubmed 出版商
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Weiss A, Roscic A, Paganetti P. Inducible mutant huntingtin expression in HN10 cells reproduces Huntington's disease-like neuronal dysfunction. Mol Neurodegener. 2009;4:11 pubmed 出版商
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Illuzzi J, Yerkes S, Parekh Olmedo H, Kmiec E. DNA breakage and induction of DNA damage response proteins precede the appearance of visible mutant huntingtin aggregates. J Neurosci Res. 2009;87:733-47 pubmed 出版商
Popiel H, Nagai Y, Fujikake N, Toda T. Delivery of the aggregate inhibitor peptide QBP1 into the mouse brain using PTDs and its therapeutic effect on polyglutamine disease mice. Neurosci Lett. 2009;449:87-92 pubmed 出版商
Desplats P, Lambert J, Thomas E. Functional roles for the striatal-enriched transcription factor, Bcl11b, in the control of striatal gene expression and transcriptional dysregulation in Huntington's disease. Neurobiol Dis. 2008;31:298-308 pubmed 出版商
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Wang J, Wang C, Orr A, Tydlacka S, Li S, Li X. Impaired ubiquitin-proteasome system activity in the synapses of Huntington's disease mice. J Cell Biol. 2008;180:1177-89 pubmed 出版商
Lim H, Lee S, Chu K, Joo K, Kang L, Im W, et al. Neuroprotective effect of neural stem cell-conditioned media in in vitro model of Huntington's disease. Neurosci Lett. 2008;435:175-80 pubmed 出版商
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Bacos K, Björkqvist M, Petersen A, Luts L, Maat Schieman M, Roos R, et al. Islet beta-cell area and hormone expression are unaltered in Huntington's disease. Histochem Cell Biol. 2008;129:623-9 pubmed 出版商
Doi H, Okamura K, Bauer P, Furukawa Y, Shimizu H, Kurosawa M, et al. RNA-binding protein TLS is a major nuclear aggregate-interacting protein in huntingtin exon 1 with expanded polyglutamine-expressing cells. J Biol Chem. 2008;283:6489-500 pubmed 出版商
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Van Raamsdonk J, Murphy Z, Selva D, Hamidizadeh R, Pearson J, Petersen A, et al. Testicular degeneration in Huntington disease. Neurobiol Dis. 2007;26:512-20 pubmed
Schilling G, Klevytska A, Tebbenkamp A, Juenemann K, Cooper J, Gonzales V, et al. Characterization of huntingtin pathologic fragments in human Huntington disease, transgenic mice, and cell models. J Neuropathol Exp Neurol. 2007;66:313-20 pubmed
Perrin V, Regulier E, Abbas Terki T, Hassig R, Brouillet E, Aebischer P, et al. Neuroprotection by Hsp104 and Hsp27 in lentiviral-based rat models of Huntington's disease. Mol Ther. 2007;15:903-11 pubmed
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Diaz Hernandez M, Torres Peraza J, Salvatori Abarca A, Morán M, Gómez Ramos P, Alberch J, et al. Full motor recovery despite striatal neuron loss and formation of irreversible amyloid-like inclusions in a conditional mouse model of Huntington's disease. J Neurosci. 2005;25:9773-81 pubmed
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产品信息
目录代码 :
MAB5374
亚类别 :
神经科学
产品名称 :
Anti-Huntingtin Protein Antibody, clone mEM48
产品类型 :
抗体
克隆性 :
单克隆抗体
基因编号 :
P42858
宿主名称 :
小鼠
抗原 :
Huntingtin Protein
克隆 :
mEM48
共轭标签 :
Culture Supernatant
抗体亚型 :
IgG
产品描述 :
Anti-Huntingtin Protein Antibody, clone mEM48
交叉活性 :
人类;大鼠;小鼠
背景 :
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.
别名 :
Huntingtin
免疫原 :
GST fusion protein from the first 256 amino acids from human huntingtin with the deletion of the polyglutamine tract.
特异性 :
Reacts with human huntingtin protein (both native and recombinant protein). MAB5374 reacts with mutant huntingtin in patients and in transgenic animals that express different numbers of repeats (from 82 to 150 glutamines). Thus, it should recognize different forms of mutant huntingtin.
包装尺寸 :
100微升
用途 :
免疫细胞化学;免疫组化;免疫印迹
储存 :
Stable for 6 months 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.
更多信息或购买 :
公司信息
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http://www.millipore.com/china/wwcn/cnhome
400-889-1988
公司总部: 美国
EMD Millipore is the Life Science division of Merck KGaA of Darmstadt, Germany

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