Published in J Neurochem on May 01, 1999
Huntingtin and huntingtin-associated protein 1 influence neuronal calcium signaling mediated by inositol-(1,4,5) triphosphate receptor type 1. Neuron (2003) 3.25
Disturbed Ca2+ signaling and apoptosis of medium spiny neurons in Huntington's disease. Proc Natl Acad Sci U S A (2005) 2.15
Calpain-mediated signaling mechanisms in neuronal injury and neurodegeneration. Mol Neurobiol (2008) 1.93
Pathophysiology of Huntington's disease: time-dependent alterations in synaptic and receptor function. Neuroscience (2011) 1.62
Dopaminergic signaling and striatal neurodegeneration in Huntington's disease. J Neurosci (2007) 1.44
NR2A and NR2B receptor gene variations modify age at onset in Huntington disease. Neurogenetics (2004) 1.31
Neuronal store-operated calcium entry pathway as a novel therapeutic target for Huntington's disease treatment. Chem Biol (2011) 1.25
Full length mutant huntingtin is required for altered Ca2+ signaling and apoptosis of striatal neurons in the YAC mouse model of Huntington's disease. Neurobiol Dis (2008) 1.12
Role of inositol 1,4,5-trisphosphate receptors in pathogenesis of Huntington's disease and spinocerebellar ataxias. Neurochem Res (2011) 1.04
Dopamine and glutamate in Huntington's disease: A balancing act. CNS Neurosci Ther (2010) 1.03
Control of excitatory synaptic transmission by C-terminal Src kinase. J Biol Chem (2008) 1.03
Differences in excitatory transmission between thalamic and cortical afferents to single spiny efferent neurons of rat dorsal striatum. Eur J Neurosci (2008) 0.98
Neuroprotective effects of a novel kynurenic acid analogue in a transgenic mouse model of Huntington's disease. J Neural Transm (Vienna) (2010) 0.96
Calpain and STriatal-Enriched protein tyrosine phosphatase (STEP) activation contribute to extrasynaptic NMDA receptor localization in a Huntington's disease mouse model. Hum Mol Genet (2012) 0.95
Selective subunit antagonists suggest an inhibitory relationship between NR2B and NR2A-subunit containing N-methyl-D: -aspartate receptors in hippocampal slices. Exp Brain Res (2004) 0.94
Ginsenosides protect striatal neurons in a cellular model of Huntington's disease. J Neurosci Res (2009) 0.94
Tetrabenazine is neuroprotective in Huntington's disease mice. Mol Neurodegener (2010) 0.93
Chronic Glutamate Toxicity in Neurodegenerative Diseases-What is the Evidence? Front Neurosci (2015) 0.92
The paradigm of Huntington's disease: therapeutic opportunities in neurodegeneration. NeuroRx (2004) 0.91
Corticostriatal circuit dysfunction in Huntington's disease: intersection of glutamate, dopamine and calcium. Future Neurol (2010) 0.89
HD iPSC-derived neural progenitors accumulate in culture and are susceptible to BDNF withdrawal due to glutamate toxicity. Hum Mol Genet (2015) 0.85
Ion channels as drug targets in central nervous system disorders. Curr Med Chem (2013) 0.84
Endogenous neuroprotection in chronic neurodegenerative disorders: with particular regard to the kynurenines. J Cell Mol Med (2011) 0.83
Effects of mutant huntingtin on mGluR5-mediated dual signaling pathways: implications for therapeutic interventions. Cell Mol Neurobiol (2010) 0.80
Somatostatin receptor 1 and 5 double knockout mice mimic neurochemical changes of Huntington's disease transgenic mice. PLoS One (2011) 0.79
Ca(2+) signaling: an outlook on the characterization of Ca(2+) channels and their importance in cellular functions. Adv Exp Med Biol (2012) 0.79
Lack of efficacy of NMDA receptor-NR2B selective antagonists in the R6/2 model of Huntington disease. Exp Neurol (2010) 0.78
Electron Transport Disturbances and Neurodegeneration: From Albert Szent-Györgyi's Concept (Szeged) till Novel Approaches to Boost Mitochondrial Bioenergetics. Oxid Med Cell Longev (2015) 0.75
Inositol 1,4,5-tripshosphate receptor, calcium signaling, and polyglutamine expansion disorders. Curr Top Membr (2010) 0.75
Levodopa-Induced Dyskinesia Is Related to Indirect Pathway Medium Spiny Neuron Excitotoxicity: A Hypothesis Based on an Unexpected Finding. Parkinsons Dis (2016) 0.75
Selective A2A receptor antagonist SCH 58261 modulates striatal oxidative stress and alleviates toxicity induced by 3-Nitropropionic acid in male Wistar rats. Metab Brain Dis (2017) 0.75
Mutations in ABC1 in Tangier disease and familial high-density lipoprotein deficiency. Nat Genet (1999) 7.30
The psychological consequences of predictive testing for Huntington's disease. Canadian Collaborative Study of Predictive Testing. N Engl J Med (1992) 5.51
Loss of huntingtin-mediated BDNF gene transcription in Huntington's disease. Science (2001) 5.28
A new model for prediction of the age of onset and penetrance for Huntington's disease based on CAG length. Clin Genet (2004) 4.73
A highly polymorphic locus very tightly linked to the Huntington's disease gene. Nature (1988) 3.90
A gene encoding a putative GTPase regulator is mutated in familial amyotrophic lateral sclerosis 2. Nat Genet (2001) 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
Targeted disruption of the Huntington's disease gene results in embryonic lethality and behavioral and morphological changes in heterozygotes. Cell (1995) 3.45
Implementing community-based participatory research centers in diverse urban settings. J Urban Health (2001) 3.37
CAG repeat expansion in Huntington disease determines age at onset in a fully dominant fashion. Neurology (2012) 3.12
Cleavage of huntingtin by apopain, a proapoptotic cysteine protease, is modulated by the polyglutamine tract. Nat Genet (1996) 3.08
Phenotypic characterization of individuals with 30-40 CAG repeats in the Huntington disease (HD) gene reveals HD cases with 36 repeats and apparently normal elderly individuals with 36-39 repeats. Am J Hum Genet (1996) 2.88
The likelihood of being affected with Huntington disease by a particular age, for a specific CAG size. Am J Hum Genet (1997) 2.84
Replication of TPMT and ABCC3 genetic variants highly associated with cisplatin-induced hearing loss in children. Clin Pharmacol Ther (2013) 2.70
A one-hit model of cell death in inherited neuronal degenerations. Nature (2000) 2.65
Predictive testing for Huntington disease in Canada: the experience of those receiving an increased risk. Am J Med Genet (1992) 2.53
Length of huntingtin and its polyglutamine tract influences localization and frequency of intracellular aggregates. Nat Genet (1998) 2.42
Retracted Infectious measles virus from cloned cDNA. EMBO J (1990) 2.36
Pharmacogenetics of neonatal opioid toxicity following maternal use of codeine during breastfeeding: a case-control study. Clin Pharmacol Ther (2008) 2.31
Caspase cleavage of gene products associated with triplet expansion disorders generates truncated fragments containing the polyglutamine tract. J Biol Chem (1998) 2.23
Characterization and organization of DNA sequences adjacent to the human telomere associated repeat (TTAGGG)n. Nucleic Acids Res (1990) 2.23
Deletion of Huntington's disease-linked G8 (D4S10) locus in Wolf-Hirschhorn syndrome. Nature (1985) 2.19
Loss-of-function mutations in the Nav1.7 gene underlie congenital indifference to pain in multiple human populations. Clin Genet (2007) 2.15
A worldwide assessment of the frequency of suicide, suicide attempts, or psychiatric hospitalization after predictive testing for Huntington disease. Am J Hum Genet (1999) 2.12
Clinical markers of early disease in persons near onset of Huntington's disease. Neurology (2001) 2.11
An actin-binding protein of the Sla2/Huntingtin interacting protein 1 family is a novel component of clathrin-coated pits and vesicles. J Cell Biol (1999) 2.10
HIP1, a human homologue of S. cerevisiae Sla2p, interacts with membrane-associated huntingtin in the brain. Nat Genet (1997) 2.09
Mutations in the ABC1 gene in familial HDL deficiency with defective cholesterol efflux. Lancet (1999) 2.02
Age and residual cholesterol efflux affect HDL cholesterol levels and coronary artery disease in ABCA1 heterozygotes. J Clin Invest (2000) 1.98
A CCG repeat polymorphism adjacent to the CAG repeat in the Huntington disease gene: implications for diagnostic accuracy and predictive testing. Hum Mol Genet (1994) 1.97
Wild-type huntingtin protects from apoptosis upstream of caspase-3. J Neurosci (2000) 1.92
Molecular cloning and characterization of human tissue inhibitor of metalloproteinase 4. J Biol Chem (1996) 1.92
The influence of huntingtin protein size on nuclear localization and cellular toxicity. J Cell Biol (1998) 1.87
Cholesterol efflux via ATP-binding cassette transporter A1 (ABCA1) and cholesterol uptake via the LDL receptor influences cholesterol-induced impairment of beta cell function in mice. Diabetologia (2010) 1.87
Contribution of DNA sequence and CAG size to mutation frequencies of intermediate alleles for Huntington disease: evidence from single sperm analyses. Hum Mol Genet (1997) 1.82
Inhibiting caspase cleavage of huntingtin reduces toxicity and aggregate formation in neuronal and nonneuronal cells. J Biol Chem (2000) 1.79
Subtype-dependence of NMDA receptor channel open probability. J Neurosci (1999) 1.77
Cell death attenuation by 'Usurpin', a mammalian DED-caspase homologue that precludes caspase-8 recruitment and activation by the CD-95 (Fas, APO-1) receptor complex. Cell Death Differ (1998) 1.76
NMDA receptor function in mouse models of Huntington disease. J Neurosci Res (2001) 1.76
Opinion: predictive testing for Huntington disease in childhood: challenges and implications. Am J Hum Genet (1990) 1.75
Common genetic variation in ABCA1 is associated with altered lipoprotein levels and a modified risk for coronary artery disease. Circulation (2001) 1.71
First-trimester prenatal diagnosis for Huntington's disease with DNA probes. Lancet (1987) 1.70
Kennedy's disease: caspase cleavage of the androgen receptor is a crucial event in cytotoxicity. J Neurochem (1999) 1.66
Validation of variants in SLC28A3 and UGT1A6 as genetic markers predictive of anthracycline-induced cardiotoxicity in children. Pediatr Blood Cancer (2013) 1.65
Lipoprotein lipase activity is associated with severity of angina pectoris. REGRESS Study Group. Circulation (2000) 1.65
Evidence from family studies that the gene causing Huntington disease is telomeric to D4S95 and D4S90. Am J Hum Genet (1989) 1.64
Soccerball-induced eye injuries. JAMA (1983) 1.63
Pivotal role of ABCA1 in reverse cholesterol transport influencing HDL levels and susceptibility to atherosclerosis. J Lipid Res (2001) 1.62
DNA polymorphisms in and around the Apo-A1-CIII genes and genetic hyperlipidemias. Am J Hum Genet (1987) 1.61
Differential effect of the rs4149056 variant in SLCO1B1 on myopathy associated with simvastatin and atorvastatin. Pharmacogenomics J (2011) 1.60
Diagnosis of Huntington disease: a model for the stages of psychological response based on experience of a predictive testing program. Am J Med Genet (1993) 1.59
Predictive, pre-natal and diagnostic genetic testing for Huntington's disease: the experience in Canada from 1987 to 2000. Clin Genet (2003) 1.58
Developmentally regulated plant genes: the nucleotide sequence of a wheat gliadin genomic clone. EMBO J (1984) 1.57
Increased instability of intermediate alleles in families with sporadic Huntington disease compared to similar sized intermediate alleles in the general population. Hum Mol Genet (1995) 1.55
HIP1 functions in clathrin-mediated endocytosis through binding to clathrin and adaptor protein 2. J Biol Chem (2001) 1.54
Regulation of ligand-gated ion channels by protein phosphorylation. Adv Second Messenger Phosphoprotein Res (1999) 1.54
CDC Urban Research Centers: community-based participatory research to improve the health of urban communities. J Womens Health Gend Based Med (2001) 1.54
Expanded polyglutamines in Caenorhabditis elegans cause axonal abnormalities and severe dysfunction of PLM mechanosensory neurons without cell death. Proc Natl Acad Sci U S A (2001) 1.53
The Gln-Ala repeat transcriptional activator CA150 interacts with huntingtin: neuropathologic and genetic evidence for a role in Huntington's disease pathogenesis. Proc Natl Acad Sci U S A (2001) 1.51
Multicenter clinical trial of recombinant human insulin-like growth factor I in patients with acute renal failure. Kidney Int (1999) 1.51
Huntingtin is ubiquitinated and interacts with a specific ubiquitin-conjugating enzyme. J Biol Chem (1996) 1.47
Resonant dissociative electron transfer of the presolvated electron to CCl4 in liquid: Direct observation and lifetime of the CCl4*- transition state. J Chem Phys (2008) 1.46
Genetic linkage between Huntington disease and the D4S10 locus in South African families: further evidence against non-allelic heterogeneity. Hum Genet (1991) 1.46
Predictive testing for Huntington disease: II. Demographic characteristics, life-style patterns, attitudes, and psychosocial assessments of the first fifty-one test candidates. Am J Med Genet (1989) 1.46
The raccoon ascarid. A probable cause of human ocular larva migrans. Ophthalmology (1985) 1.45
Specific caspase interactions and amplification are involved in selective neuronal vulnerability in Huntington's disease. Cell Death Differ (2004) 1.44
Communicating pharmacogenetic research results to breastfeeding mothers taking codeine: a pilot study of perceptions and benefits. Clin Pharmacol Ther (2010) 1.44
Sex-dependent mechanisms for expansions and contractions of the CAG repeat on affected Huntington disease chromosomes. Am J Hum Genet (1995) 1.43
A somatic cell hybrid panel for localizing DNA segments near the Huntington's disease gene. Genomics (1987) 1.42
A major insertion accounts for a significant proportion of mutations underlying human lipoprotein lipase deficiency. Proc Natl Acad Sci U S A (1989) 1.41
Reactions of trichloroethylene epoxide in aqueous systems. Biochem Pharmacol (1979) 1.41
Perinatal and first year follow-up of patients with Prader-Willi syndrome: normal size of hands and feet. Clin Genet (1989) 1.41
Stress activation of Bacillus subtilis sigma B can occur in the absence of the sigma B negative regulator RsbX. J Bacteriol (1997) 1.41
Identification of an autonomously initiating RNA polymerase III holoenzyme containing a novel factor that is selectively inactivated during protein synthesis inhibition. Genes Dev (1997) 1.40
Measurement of mutational flow implies both a high new-mutation rate for Huntington disease and substantial underascertainment of late-onset cases. Am J Hum Genet (2001) 1.39
A PCR method for accurate assessment of trinucleotide repeat expansion in Huntington disease. Hum Mol Genet (1993) 1.39
George Huntington: the man behind the eponym. J Med Genet (1993) 1.39
Wild-type huntingtin reduces the cellular toxicity of mutant huntingtin in vivo. Am J Hum Genet (2000) 1.38
Positron emission tomography in the early diagnosis of Huntington's disease. Neurology (1986) 1.33
The estrogenicity of bisphenol A-related diphenylalkanes with various substituents at the central carbon and the hydroxy groups. Environ Health Perspect (1998) 1.33
Differential sensitivity of recombinant N-methyl-D-aspartate receptor subtypes to zinc inhibition. Mol Pharmacol (1997) 1.33
Apolipoprotein B gene variants are involved in the determination of serum cholesterol levels: a study in normo- and hyperlipidaemic individuals. Atherosclerosis (1987) 1.32
Partial gene duplication involving exon-Alu interchange results in lipoprotein lipase deficiency. Am J Hum Genet (1990) 1.31
Predictive testing for Huntington disease: interpretation and significance of intermediate alleles. Clin Genet (2006) 1.29
Human ABCA1 BAC transgenic mice show increased high density lipoprotein cholesterol and ApoAI-dependent efflux stimulated by an internal promoter containing liver X receptor response elements in intron 1. J Biol Chem (2001) 1.28
Post-transcriptional regulation of extracellular matrix metalloproteinase in human heart end-stage failure secondary to ischemic cardiomyopathy. J Mol Cell Cardiol (1996) 1.27