Polyglutamine expansions cause decreased CRE-mediated transcription and early gene expression changes prior to cell death in an inducible cell model of Huntington's disease.

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Published in Hum Mol Genet on August 15, 2001

Authors

A Wyttenbach1, J Swartz, H Kita, T Thykjaer, J Carmichael, J Bradley, R Brown, M Maxwell, A Schapira, T F Orntoft, K Kato, D C Rubinsztein

Author Affiliations

1: Wellcome Trust Centre for Molecular Mechanisms in Disease, Cambridge Institute for Medical Research, Wellcome Trust/MRC Building, Addenbrooke's Hospital, Hills Road, Cambridge CB2 2XY, UK.

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Evaluation of a tetrazolium-based semiautomated colorimetric assay: assessment of chemosensitivity testing. Cancer Res (1987) 10.80

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Molecular definitions of cell death subroutines: recommendations of the Nomenclature Committee on Cell Death 2012. Cell Death Differ (2011) 9.44

Synthesis of infectious poliovirus RNA by purified T7 RNA polymerase. Proc Natl Acad Sci U S A (1986) 8.59

A comparison of a Patient Enablement Instrument (PEI) against two established satisfaction scales as an outcome measure of primary care consultations. Fam Pract (1998) 6.37

Intestinal mucosal lesion in low-flow states. I. A morphological, hemodynamic, and metabolic reappraisal. Arch Surg (1970) 5.62

Y-chromosomal diversity in Europe is clinal and influenced primarily by geography, rather than by language. Am J Hum Genet (2000) 4.72

Interleukin 2 high-affinity receptor expression requires two distinct binding proteins. J Exp Med (1987) 4.53

Comparison of pharmacokinetics and systemic effects of inhaled fluticasone propionate in patients with asthma and healthy volunteers: a randomised crossover study. Lancet (2000) 4.22

A model for replication repair in mammalian cells. J Mol Biol (1976) 3.78

Endogenous tumor necrosis factor (cachectin) is essential to host resistance against Listeria monocytogenes infection. Infect Immun (1988) 3.70

Topotecan versus paclitaxel for the treatment of recurrent epithelial ovarian cancer. J Clin Oncol (1997) 3.61

Insulin-regulatable tissues express a unique insulin-sensitive glucose transport protein. Nature (1988) 3.57

Glycogen-Storage Disease: Thesaurismosis Glycogenica (von Gierke). Am J Pathol (1934) 3.49

Topotecan versus cyclophosphamide, doxorubicin, and vincristine for the treatment of recurrent small-cell lung cancer. J Clin Oncol (1999) 3.48

Guidelines for the use and interpretation of assays for monitoring cell death in higher eukaryotes. Cell Death Differ (2009) 3.44

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Nationalism and patriotism: national identification and out-group rejection. Br J Soc Psychol (2001) 3.33

Case-control study of leukaemia and non-Hodgkin's lymphoma in children in Caithness near the Dounreay nuclear installation. BMJ (1991) 3.28

Revision of consensus sequence of human Alu repeats--a review. Gene (1987) 3.22

Cloning of a bcl-2 homologue by interaction with adenovirus E1B 19K. Nature (1995) 3.22

Inactivation of the N-CAM gene in mice results in size reduction of the olfactory bulb and deficits in spatial learning. Nature (1994) 3.19

Developing a 'consultation quality index' (CQI) for use in general practice. Fam Pract (2000) 3.13

Poliovirus host range is determined by a short amino acid sequence in neutralization antigenic site I. Science (1988) 3.03

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Trauma care regionalization: a process-outcome evaluation. J Trauma (1999) 3.02

Molecular cloning of the human cholecystokinin gene by use of a synthetic probe containing deoxyinosine. Proc Natl Acad Sci U S A (1985) 3.01

11beta-hydroxysteroid dehydrogenase type 1 knockout mice show attenuated glucocorticoid-inducible responses and resist hyperglycemia on obesity or stress. Proc Natl Acad Sci U S A (1997) 3.00

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Allelic variation at the VRN-1 promoter region in polyploid wheat. Theor Appl Genet (2004) 2.61

Evaluation of a tetrazolium-based semiautomated colorimetric assay: assessment of radiosensitivity. Cancer Res (1987) 2.56

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The evolution of Titan's mid-latitude clouds. Science (2005) 2.46

The international spread of methicillin-resistant Staphylococcus aureus. J Hosp Infect (1987) 2.46

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Human N-ras: cDNA cloning and gene structure. Nucleic Acids Res (1985) 2.41

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Treatment of advanced breast cancer with sterically stabilized liposomal doxorubicin: results of a multicenter phase II trial. J Clin Oncol (1997) 2.39

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Use of complementary therapies for arthritis among patients of rheumatologists. Ann Intern Med (1999) 2.35

An evaluation of the Gaspak system in the culture of anaerobic bacteria. J Appl Bacteriol (1972) 2.33

Daily walking combined with diet therapy is a useful means for obese NIDDM patients not only to reduce body weight but also to improve insulin sensitivity. Diabetes Care (1995) 2.33

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Platelet-derived growth factor in idiopathic pulmonary fibrosis. J Clin Invest (1990) 2.29

Prope tolerance, perioperative campath 1H, and low-dose cyclosporin monotherapy in renal allograft recipients. Lancet (1998) 2.26

Comparison of short-term and continuous chemotherapy (mitozantrone) for advanced breast cancer. Lancet (1990) 2.25

Hypoglycaemia in paediatric admissions in Mozambique. Lancet (1994) 2.23

Frequent beta-catenin mutation and cytoplasmic/nuclear accumulation in pancreatic solid-pseudopapillary neoplasm. Cancer Res (2001) 2.22

Phase I study of bryostatin 1: assessment of interleukin 6 and tumor necrosis factor alpha induction in vivo. The Cancer Research Campaign Phase I Committee. J Natl Cancer Inst (1993) 2.22

The Golgi association of endothelial nitric oxide synthase is necessary for the efficient synthesis of nitric oxide. J Biol Chem (1995) 2.20

Symptoms of depression and survival experience among three samples of smokers trying to quit. Psychol Addict Behav (2001) 2.20

Microsatellites show mutational bias and heterozygote instability. Nat Genet (1996) 2.18

Reversal of drug resistance in human tumor xenografts by 2'-deoxy-5-azacytidine-induced demethylation of the hMLH1 gene promoter. Cancer Res (2000) 2.17

Intramuscular rocuronium in infants and children. Dose-ranging and tracheal intubating conditions. Anesthesiology (1996) 2.14

Giant negative thermal expansion in magnetic nanocrystals. Nat Nanotechnol (2008) 2.14

Differential gene expression in colon cancer of the caecum versus the sigmoid and rectosigmoid. Gut (2005) 2.13

Loss of bacterial diversity during antibiotic treatment of intubated patients colonized with Pseudomonas aeruginosa. J Clin Microbiol (2007) 2.12

Cause of death in rheumatoid arthritis. Br J Rheumatol (1984) 2.12

Properties of gastric smooth muscles obtained from mice which lack inositol trisphosphate receptor. J Physiol (2000) 2.11

Measuring quality in general practice. Pilot study of a needs, process and outcome measure. Occas Pap R Coll Gen Pract (1997) 2.10

Insuring Latinos against the costs of illness. JAMA (1993) 2.09

Care of patients with selected health problems in fundholding practices in Scotland in 1990 and 1992: needs, process and outcome. Br J Gen Pract (1995) 2.07

Changes in gastric acid secretion assayed by endoscopic gastrin test before and after Helicobacter pylori eradication. Gut (2000) 2.06

Purified poloxamer 188 for treatment of acute vaso-occlusive crisis of sickle cell disease: A randomized controlled trial. JAMA (2001) 2.06

Transfection of CD14 into 70Z/3 cells dramatically enhances the sensitivity to complexes of lipopolysaccharide (LPS) and LPS binding protein. J Exp Med (1992) 2.05

Peptide sequences for sucrose splitting and glucan binding within Streptococcus sobrinus glucosyltransferase (water-insoluble glucan synthetase). J Bacteriol (1991) 2.05

The effect of weight reduction on blood pressure, plasma renin activity, and plasma aldosterone levels in obese patients. N Engl J Med (1981) 2.05

Excitatory cortical inputs to pallidal neurons via the subthalamic nucleus in the monkey. J Neurophysiol (2000) 2.03

A pharmacodynamic explanation for the rapid onset/offset of rapacuronium bromide. Anesthesiology (1999) 2.03

Mechanisms of heading perception in primate visual cortex. Science (1996) 2.03

Intraoral minor salivary gland tumors: a clinicopathological study of 82 cases. Int J Oral Maxillofac Surg (2005) 2.02

Cisplatin and adriamycin resistance are associated with MutLalpha and mismatch repair deficiency in an ovarian tumor cell line. J Biol Chem (1996) 2.01

Second cancers following radiation treatment for cervical cancer. An international collaboration among cancer registries. J Natl Cancer Inst (1985) 2.00

New cosmid vectors developed for eukaryotic DNA cloning. Gene (1984) 1.97

Acute infectious epiglottitis in adults. Ann Intern Med (1969) 1.97

Characterization of a broadly expressed human leucocyte surface antigen MEM-43 anchored in membrane through phosphatidylinositol. Mol Immunol (1989) 1.94

The native rat olfactory cyclic nucleotide-gated channel is composed of three distinct subunits. J Neurosci (1999) 1.93

Mutations in SIP1, encoding Smad interacting protein-1, cause a form of Hirschsprung disease. Nat Genet (2001) 1.93

Training in international animal health. Vet Rec (2001) 1.93

EXPERIENCES WITH SODIUM AMYTAL AS A GENERAL ANAESTHETIC. Ann Surg (1930) 1.93

A death-domain-containing receptor that mediates apoptosis. Nature (1996) 1.92

A molecular investigation of true dominance in Huntington's disease. J Med Genet (1999) 1.92