Published in Nature on May 20, 1999
Coenzyme Q10 in Huntington's Disease (HD) (2CARE) | NCT00608881
Pyroptosis: host cell death and inflammation. Nat Rev Microbiol (2009) 7.63
RNA interference improves motor and neuropathological abnormalities in a Huntington's disease mouse model. Proc Natl Acad Sci U S A (2005) 4.79
Accumulation of mutant huntingtin fragments in aggresome-like inclusion bodies as a result of insufficient protein degradation. Mol Biol Cell (2001) 3.41
Mechanisms of apoptosis. Am J Pathol (2000) 3.23
Anthrax lethal toxin and Salmonella elicit the common cell death pathway of caspase-1-dependent pyroptosis via distinct mechanisms. Proc Natl Acad Sci U S A (2008) 2.82
Polyglutamine aggregates alter protein folding homeostasis in Caenorhabditis elegans. Proc Natl Acad Sci U S A (2000) 2.63
Cell death in the nervous system. Nature (2006) 2.62
Proteases for cell suicide: functions and regulation of caspases. Microbiol Mol Biol Rev (2000) 2.48
Dietary restriction normalizes glucose metabolism and BDNF levels, slows disease progression, and increases survival in huntingtin mutant mice. Proc Natl Acad Sci U S A (2003) 2.35
Minocycline inhibits caspase-independent and -dependent mitochondrial cell death pathways in models of Huntington's disease. Proc Natl Acad Sci U S A (2003) 2.05
Inhibition of huntingtin fibrillogenesis by specific antibodies and small molecules: implications for Huntington's disease therapy. Proc Natl Acad Sci U S A (2000) 1.88
Inhibitors of protein disulfide isomerase suppress apoptosis induced by misfolded proteins. Nat Chem Biol (2010) 1.62
Minocycline: far beyond an antibiotic. Br J Pharmacol (2013) 1.59
Histone deacetylase inhibitors for treating a spectrum of diseases not related to cancer. Mol Med (2011) 1.41
Fundamental role of the Rip2/caspase-1 pathway in hypoxia and ischemia-induced neuronal cell death. Proc Natl Acad Sci U S A (2003) 1.40
Harnessing chaperone-mediated autophagy for the selective degradation of mutant huntingtin protein. Nat Biotechnol (2010) 1.38
Protease-activated drug development. Theranostics (2012) 1.36
Multiple pathways contribute to the pathogenesis of Huntington disease. Mol Neurodegener (2006) 1.35
Phosphorylation of threonine 3: implications for Huntingtin aggregation and neurotoxicity. J Biol Chem (2009) 1.34
Expression of Huntington's disease protein results in apoptotic neurons in the brains of cloned transgenic pigs. Hum Mol Genet (2010) 1.33
The use of the R6 transgenic mouse models of Huntington's disease in attempts to develop novel therapeutic strategies. NeuroRx (2005) 1.33
Tauroursodeoxycholic acid, a bile acid, is neuroprotective in a transgenic animal model of Huntington's disease. Proc Natl Acad Sci U S A (2002) 1.29
The role of the inflammasome in nonmyeloid cells. J Clin Immunol (2010) 1.18
Inhibitors of cytochrome c release with therapeutic potential for Huntington's disease. J Neurosci (2008) 1.17
Huntingtin inhibits caspase-3 activation. EMBO J (2006) 1.17
Huntington's disease. Mol Pathol (2001) 1.16
Huntingtin in health and disease. J Clin Invest (2003) 1.14
Polyglutamine disease and neuronal cell death. Proc Natl Acad Sci U S A (2000) 1.14
Neurodegeneration in Alzheimer's disease: caspases and synaptic element interdependence. Mol Neurodegener (2009) 1.12
Drosophila models of neurodegenerative disease. NeuroRx (2005) 1.12
An AAV-derived Apaf-1 dominant negative inhibitor prevents MPTP toxicity as antiapoptotic gene therapy for Parkinson's disease. Proc Natl Acad Sci U S A (2001) 1.11
Polyglutamine-expanded androgen receptor truncation fragments activate a Bax-dependent apoptotic cascade mediated by DP5/Hrk. J Neurosci (2009) 1.10
Tumor necrosis factor-related apoptosis-inducing ligand induces caspase-dependent interleukin-8 expression and apoptosis in human astroglioma cells. Mol Cell Biol (2002) 1.10
Autophagy and neuronal cell death in neurological disorders. Cold Spring Harb Perspect Biol (2012) 1.08
Late-onset neurodegenerative diseases--the role of protein insolubility. J Anat (2000) 1.07
Translating therapies for Huntington's disease from genetic animal models to clinical trials. NeuroRx (2004) 1.05
Inhibition of mitochondrial protein import by mutant huntingtin. Nat Neurosci (2014) 1.04
The role of the immune system in Huntington's disease. Clin Dev Immunol (2013) 1.03
The executioners sing a new song: killer caspases activate microglia. Cell Death Differ (2011) 1.02
Brain tissue responses to neural implants impact signal sensitivity and intervention strategies. ACS Chem Neurosci (2015) 0.99
Genetics and neuropathology of Huntington's disease. Int Rev Neurobiol (2011) 0.99
Caspases as therapeutic targets in Alzheimer's disease: is it time to "cut" to the chase? Int J Clin Exp Pathol (2008) 0.97
The melatonin MT1 receptor axis modulates mutant Huntingtin-mediated toxicity. J Neurosci (2011) 0.96
Minocycline as a potential therapeutic agent in neurodegenerative disorders characterised by protein misfolding. Prion (2009) 0.94
The berkeleyacetals, three meroterpenes from a deep water acid mine waste Penicillium. J Nat Prod (2007) 0.94
The tryptophan metabolite 3-hydroxyanthranilic acid plays anti-inflammatory and neuroprotective roles during inflammation: role of hemeoxygenase-1. Am J Pathol (2011) 0.93
Increased caspase-2, calpain activations and decreased mitochondrial complex II activity in cells expressing exogenous huntingtin exon 1 containing CAG repeat in the pathogenic range. Cell Mol Neurobiol (2007) 0.93
Intracerebral transplantation of neural stem cells combined with trehalose ingestion alleviates pathology in a mouse model of Huntington's disease. J Neurosci Res (2009) 0.92
Inhibition of trypsin-like cysteine proteinases (gingipains) from Porphyromonas gingivalis by tetracycline and its analogues. Antimicrob Agents Chemother (2001) 0.92
Meclizine is neuroprotective in models of Huntington's disease. Hum Mol Genet (2010) 0.92
Effects of caspase-1 knockout on chronic neural recording quality and longevity: insight into cellular and molecular mechanisms of the reactive tissue response. Biomaterials (2014) 0.92
The paradigm of Huntington's disease: therapeutic opportunities in neurodegeneration. NeuroRx (2004) 0.91
Minimal role for caspase 12 in the unfolded protein response in oligodendrocytes in vivo. J Neurochem (2007) 0.91
FE65 binds Teashirt, inhibiting expression of the primate-specific caspase-4. PLoS One (2009) 0.91
Caspases as therapeutic targets. J Cell Mol Med (2008) 0.89
Unexpected off-targeting effects of anti-huntingtin ribozymes and siRNA in vivo. Neurobiol Dis (2007) 0.89
The role of IκB kinase complex in the neurobiology of Huntington's disease. Neurobiol Dis (2011) 0.87
Defective signal transduction in B lymphocytes lacking presenilin proteins. Proc Natl Acad Sci U S A (2008) 0.86
Mouse models of polyglutamine diseases in therapeutic approaches: review and data table. Part II. Mol Neurobiol (2012) 0.86
Neuroprotective effects of M826, a reversible caspase-3 inhibitor, in the rat malonate model of Huntington's disease. Br J Pharmacol (2004) 0.86
Transgenic mice neuronally expressing baculoviral p35 are resistant to diverse types of induced apoptosis, including seizure-associated neurodegeneration. Proc Natl Acad Sci U S A (2000) 0.86
The role of the immune system in triplet repeat expansion diseases. Mediators Inflamm (2015) 0.85
A huntingtin peptide inhibits polyQ-huntingtin associated defects. PLoS One (2013) 0.85
Therapeutic approaches to preventing cell death in Huntington disease. Prog Neurobiol (2012) 0.84
Genotype-, aging-dependent abnormal caspase activity in Huntington disease blood cells. J Neural Transm (Vienna) (2011) 0.83
Substrate and inhibitor-induced dimerization and cooperativity in caspase-1 but not caspase-3. J Biol Chem (2013) 0.82
Huntington's Disease: From Mutant Huntingtin Protein to Neurotrophic Factor Therapy. Int J Biomed Sci (2011) 0.82
From pathways to targets: understanding the mechanisms behind polyglutamine disease. Biomed Res Int (2014) 0.81
Ataxin-3 protein and RNA toxicity in spinocerebellar ataxia type 3: current insights and emerging therapeutic strategies. Mol Neurobiol (2013) 0.81
Activated microglia proliferate at neurites of mutant huntingtin-expressing neurons. Neurobiol Aging (2011) 0.81
Caspase activation in transgenic mice with Alzheimer-like pathology: results from a pilot study utilizing the caspase inhibitor, Q-VD-OPh. Int J Clin Exp Med (2009) 0.81
Caspase-1 inhibitors from an extremophilic fungus that target specific leukemia cell lines. J Nat Prod (2012) 0.80
Apoptosis. Dead end for neurodegeneration? Nature (1999) 0.80
Gene delivery of antioxidant enzymes inhibits human immunodeficiency virus type 1 gp120-induced expression of caspases. Neuroscience (2012) 0.80
Programmed cell death and its role in inflammation. Mil Med Res (2015) 0.79
Caspase-1 and -3 inhibiting drimane sesquiterpenoids from the extremophilic fungus Penicillium solitum. J Nat Prod (2012) 0.79
Multidrug resistance protein 1 reduces the aggregation of mutant huntingtin in neuronal cells derived from the Huntington's disease R6/2 model. Sci Rep (2015) 0.76
Novel therapies in the search for a cure for Huntington's disease. Proc Natl Acad Sci U S A (2001) 0.76
Mouse models of Machado-Joseph disease and other polyglutamine spinocerebellar ataxias. NeuroRx (2005) 0.76
The education of a brain transplant. Proc Natl Acad Sci U S A (1999) 0.75
Unraveling protein misfolding diseases using model systems. Future Sci OA (2015) 0.75
Post-translational Modifications and Protein Quality Control in Motor Neuron and Polyglutamine Diseases. Front Mol Neurosci (2017) 0.75
Chaperones in Polyglutamine Aggregation: Beyond the Q-Stretch. Front Neurosci (2017) 0.75
Of mice and men: solving the molecular mysteries of Huntington's disease. J Anat (2000) 0.75
Attenuated nuclear shrinkage in neurones with nuclear inclusions of SCA1 brains. J Neurol Neurosurg Psychiatry (2003) 0.75
An Overview of Potential Targets for Treating Amyotrophic Lateral Sclerosis and Huntington's Disease. Biomed Res Int (2015) 0.75
Cleavage of BID by caspase 8 mediates the mitochondrial damage in the Fas pathway of apoptosis. Cell (1998) 16.08
Caspase-12 mediates endoplasmic-reticulum-specific apoptosis and cytotoxicity by amyloid-beta. Nature (2000) 13.45
The functional anatomy of basal ganglia disorders. Trends Neurosci (1989) 12.28
Aggregation of huntingtin in neuronal intranuclear inclusions and dystrophic neurites in brain. Science (1997) 11.53
Classification of cell death: recommendations of the Nomenclature Committee on Cell Death 2009. Cell Death Differ (2008) 10.45
Molecular definitions of cell death subroutines: recommendations of the Nomenclature Committee on Cell Death 2012. Cell Death Differ (2011) 9.44
Neuropathological classification of Huntington's disease. J Neuropathol Exp Neurol (1985) 7.93
FcR gamma chain deletion results in pleiotrophic effector cell defects. Cell (1994) 7.66
Induction of apoptosis in fibroblasts by IL-1 beta-converting enzyme, a mammalian homolog of the C. elegans cell death gene ced-3. Cell (1993) 7.16
Human ICE/CED-3 protease nomenclature. Cell (1996) 7.11
Caveolin-1 null mice are viable but show evidence of hyperproliferative and vascular abnormalities. J Biol Chem (2001) 7.03
Cooperative regulation of cell polarity and growth by Drosophila tumor suppressors. Science (2000) 6.44
KSHV antibodies among Americans, Italians and Ugandans with and without Kaposi's sarcoma. Nat Med (1996) 5.96
Influenza A virus NS1 protein prevents activation of NF-kappaB and induction of alpha/beta interferon. J Virol (2000) 5.44
Proteases to die for. Genes Dev (1998) 5.43
Rapid induction of Alzheimer A beta amyloid formation by zinc. Science (1994) 5.34
Loss of huntingtin-mediated BDNF gene transcription in Huntington's disease. Science (2001) 5.28
Ich-1, an Ice/ced-3-related gene, encodes both positive and negative regulators of programmed cell death. Cell (1994) 5.25
Cre-mediated gene deletion in the mammary gland. Nucleic Acids Res (1997) 4.89
American College of Rheumatology classification criteria for Sjögren's syndrome: a data-driven, expert consensus approach in the Sjögren's International Collaborative Clinical Alliance cohort. Arthritis Care Res (Hoboken) (2012) 4.82
Live attenuated malaria vaccine designed to protect through hepatic CD8⁺ T cell immunity. Science (2011) 4.72
A 1.5 million-base pair inversion polymorphism in families with Williams-Beuren syndrome. Nat Genet (2001) 4.58
Dicistronic targeting constructs: reporters and modifiers of mammalian gene expression. Proc Natl Acad Sci U S A (1994) 4.44
Seroepidemiology for MERS coronavirus using microneutralisation and pseudoparticle virus neutralisation assays reveal a high prevalence of antibody in dromedary camels in Egypt, June 2013. Euro Surveill (2013) 4.37
Neurotoxicity induces cleavage of p35 to p25 by calpain. Nature (2000) 4.31
Nuclear and neuropil aggregates in Huntington's disease: relationship to neuropathology. J Neurosci (1999) 4.27
The effects of frequent nocturnal home hemodialysis: the Frequent Hemodialysis Network Nocturnal Trial. Kidney Int (2011) 4.27
Association of transcription factor APRF and protein kinase Jak1 with the interleukin-6 signal transducer gp130. Science (1994) 4.24
Minocycline inhibits caspase-1 and caspase-3 expression and delays mortality in a transgenic mouse model of Huntington disease. Nat Med (2000) 4.18
Prevention of vertebrate neuronal death by the crmA gene. Science (1994) 4.09
Tissue plasminogen activator (tPA) increases neuronal damage after focal cerebral ischemia in wild-type and tPA-deficient mice. Nat Med (1998) 4.09
CAG repeat number governs the development rate of pathology in Huntington's disease. Ann Neurol (1997) 4.01
Cross-talk between two cysteine protease families. Activation of caspase-12 by calpain in apoptosis. J Cell Biol (2000) 3.97
Tolerization of dendritic cells by T(S) cells: the crucial role of inhibitory receptors ILT3 and ILT4. Nat Immunol (2002) 3.91
A cyclic antimicrobial peptide produced in primate leukocytes by the ligation of two truncated alpha-defensins. Science (1999) 3.90
15-deoxy-delta 12,14-prostaglandin J2 inhibits multiple steps in the NF-kappa B signaling pathway. Proc Natl Acad Sci U S A (2000) 3.89
Neurological abnormalities in a knock-in mouse model of Huntington's disease. Hum Mol Genet (2001) 3.84
Array-based genomic resequencing of human leukemia. Oncogene (2010) 3.77
Huntingtin is required for neurogenesis and is not impaired by the Huntington's disease CAG expansion. Nat Genet (1997) 3.64
Murine caspase-11, an ICE-interacting protease, is essential for the activation of ICE. Cell (1998) 3.62
Apolipoprotein E epsilon 4 and cerebral hemorrhage associated with amyloid angiopathy. Ann Neurol (1995) 3.60
Homer binds a novel proline-rich motif and links group 1 metabotropic glutamate receptors with IP3 receptors. Neuron (1998) 3.59
A YAC mouse model for Huntington's disease with full-length mutant huntingtin, cytoplasmic toxicity, and selective striatal neurodegeneration. Neuron (1999) 3.50
Cellular response to endoplasmic reticulum stress: a matter of life or death. Cell Death Differ (2006) 3.50
Caspases in apoptosis and beyond. Oncogene (2008) 3.47
Novel amyloid precursor protein mutation in an Iowa family with dementia and severe cerebral amyloid angiopathy. Ann Neurol (2001) 3.46
Guidelines for the use and interpretation of assays for monitoring cell death in higher eukaryotes. Cell Death Differ (2009) 3.44
Assembly of the mitochondrial membrane system: sequences of yeast mitochondrial valine and an unusual threonine tRNA gene. Cell (1979) 3.39
A lamin B receptor in the nuclear envelope. Proc Natl Acad Sci U S A (1988) 3.28
Defects in regulation of apoptosis in caspase-2-deficient mice. Genes Dev (1998) 3.24
Crystal structure and functional analysis of the HERG potassium channel N terminus: a eukaryotic PAS domain. Cell (1998) 3.22
Functional role of caspase-1 and caspase-3 in an ALS transgenic mouse model. Science (2000) 3.12
Codon recognition rules in yeast mitochondria. Proc Natl Acad Sci U S A (1980) 3.11
Essential requirement for caspase-8/FLICE in the initiation of the Fas-induced apoptotic cascade. Curr Biol (1998) 3.11
Huntingtin is a cytoplasmic protein associated with vesicles in human and rat brain neurons. Neuron (1995) 3.08
Decreased expression of striatal signaling genes in a mouse model of Huntington's disease. Hum Mol Genet (2000) 3.07
High-dose intravenous immune globulin for stiff-person syndrome. N Engl J Med (2001) 3.04
Identification of small-molecule inhibitors of interaction between the BH3 domain and Bcl-xL. Nat Cell Biol (2001) 3.03
Office of Rare Diseases neuropathologic criteria for corticobasal degeneration. J Neuropathol Exp Neurol (2002) 3.02
Middle East Respiratory Syndrome (MERS) coronavirus seroprevalence in domestic livestock in Saudi Arabia, 2010 to 2013. Euro Surveill (2013) 2.99
Inhibition of interleukin 1beta converting enzyme family proteases reduces ischemic and excitotoxic neuronal damage. Proc Natl Acad Sci U S A (1997) 2.99
Inactivation of the mouse Huntington's disease gene homolog Hdh. Science (1995) 2.96
Evidence for in vivo but not in vitro expression of a Borrelia burgdorferi outer surface protein F (OspF) homologue. Mol Microbiol (1995) 2.92
Alcohol drinking and all cancer mortality: a meta-analysis. Ann Oncol (2012) 2.83
Comparative evaluation of the antitumor activity of antiangiogenic proteins delivered by gene transfer. Proc Natl Acad Sci U S A (2001) 2.81
Differential loss of striatal projection neurons in Huntington disease. Proc Natl Acad Sci U S A (1988) 2.78
Vascular endothelial growth factor-related protein: a ligand and specific activator of the tyrosine kinase receptor Flt4. Proc Natl Acad Sci U S A (1996) 2.77
Shigella-induced apoptosis is dependent on caspase-1 which binds to IpaB. J Biol Chem (1998) 2.75
Hrp pilus: an hrp-dependent bacterial surface appendage produced by Pseudomonas syringae pv. tomato DC3000. Proc Natl Acad Sci U S A (1997) 2.71
Identification of the paromomycin-resistance mutation in the 15 S rRNA gene of yeast mitochondria. J Biol Chem (1982) 2.70
Ribosomal protein S7 as a novel modulator of p53-MDM2 interaction: binding to MDM2, stabilization of p53 protein, and activation of p53 function. Oncogene (2007) 2.68
Long glutamine tracts cause nuclear localization of a novel form of huntingtin in medium spiny striatal neurons in HdhQ92 and HdhQ111 knock-in mice. Hum Mol Genet (2000) 2.67
Caveolin-3 null mice show a loss of caveolae, changes in the microdomain distribution of the dystrophin-glycoprotein complex, and t-tubule abnormalities. J Biol Chem (2001) 2.62
Solution structure of BID, an intracellular amplifier of apoptotic signaling. Cell (1999) 2.58
Activation and cleavage of caspase-3 in apoptosis induced by experimental cerebral ischemia. J Neurosci (1998) 2.55
Fusion of two novel genes, RBM15 and MKL1, in the t(1;22)(p13;q13) of acute megakaryoblastic leukemia. Nat Genet (2001) 2.55
The Peutz-Jegher gene product LKB1 is a mediator of p53-dependent cell death. Mol Cell (2001) 2.53
Rate of functional decline in Huntington's disease. Huntington Study Group. Neurology (2000) 2.49
Caspase-8 is required for cell death induced by expanded polyglutamine repeats. Neuron (1999) 2.41
Ketamine anesthesia during the first week of life can cause long-lasting cognitive deficits in rhesus monkeys. Neurotoxicol Teratol (2011) 2.41
Homozygotes for Huntington's disease. Nature (1987) 2.40
Deregulation of cell growth by the K1 gene of Kaposi's sarcoma-associated herpesvirus. Nat Med (1998) 2.38
Diagnosis of cerebral amyloid angiopathy. Sensitivity and specificity of cortical biopsy. Stroke (1997) 2.38
The gut as a portal of entry for bacteremia. Role of protein malnutrition. Ann Surg (1987) 2.38
Strychnine binding associated with glycine receptors of the central nervous system. Proc Natl Acad Sci U S A (1973) 2.37
A novel receptor for Apo2L/TRAIL contains a truncated death domain. Curr Biol (1997) 2.31