Published in Hum Mol Genet on April 29, 2008
IKK phosphorylates Huntingtin and targets it for degradation by the proteasome and lysosome. J Cell Biol (2009) 2.16
Proteolysis of mutant huntingtin produces an exon 1 fragment that accumulates as an aggregated protein in neuronal nuclei in Huntington disease. J Biol Chem (2010) 2.10
Mutant huntingtin N-terminal fragments of specific size mediate aggregation and toxicity in neuronal cells. J Biol Chem (2009) 1.47
Mitochondrial structural and functional dynamics in Huntington's disease. Brain Res Rev (2009) 1.37
PROSPER: an integrated feature-based tool for predicting protease substrate cleavage sites. PLoS One (2012) 1.28
Does Huntingtin play a role in selective macroautophagy? Cell Cycle (2010) 1.20
Mutant huntingtin, abnormal mitochondrial dynamics, defective axonal transport of mitochondria, and selective synaptic degeneration in Huntington's disease. Biochim Biophys Acta (2011) 1.16
Substrate-induced conformational changes occur in all cleaved forms of caspase-6. J Mol Biol (2010) 1.10
Polyglutamine-expanded androgen receptor truncation fragments activate a Bax-dependent apoptotic cascade mediated by DP5/Hrk. J Neurosci (2009) 1.10
Cleavage at the 586 amino acid caspase-6 site in mutant huntingtin influences caspase-6 activation in vivo. J Neurosci (2010) 1.10
Small changes, big impact: posttranslational modifications and function of huntingtin in Huntington disease. Neuroscientist (2011) 1.08
Mouse models of Huntington disease: variations on a theme. Dis Model Mech (2009) 1.02
Transgenic mice expressing caspase-6-derived N-terminal fragments of mutant huntingtin develop neurologic abnormalities with predominant cytoplasmic inclusion pathology composed largely of a smaller proteolytic derivative. Hum Mol Genet (2011) 1.00
Therapeutic targeting of autophagy in neurodegenerative and infectious diseases. J Exp Med (2015) 0.98
An optimized activity-based probe for the study of caspase-6 activation. Chem Biol (2012) 0.97
Transcriptional dysregulation in Huntington's disease: a failure of adaptive transcriptional homeostasis. Drug Discov Today (2014) 0.91
Genetic manipulations of mutant huntingtin in mice: new insights into Huntington's disease pathogenesis. FEBS J (2013) 0.90
SUMO-2 and PIAS1 modulate insoluble mutant huntingtin protein accumulation. Cell Rep (2013) 0.88
Novel piperazine induces apoptosis in U937 cells. Int J Biochem Mol Biol (2011) 0.84
Modulation of mutant huntingtin N-terminal cleavage and its effect on aggregation and cell death. Neurotox Res (2010) 0.83
The caspase 6 derived N-terminal fragment of DJ-1 promotes apoptosis via increased ROS production. Cell Death Differ (2012) 0.83
A whole cell assay to measure caspase-6 activity by detecting cleavage of lamin A/C. PLoS One (2012) 0.83
Cerebrospinal fluid tau cleaved by caspase-6 reflects brain levels and cognition in aging and Alzheimer disease. J Neuropathol Exp Neurol (2013) 0.81
Scalable production in human cells and biochemical characterization of full-length normal and mutant huntingtin. PLoS One (2015) 0.80
A Huntingtin-based peptide inhibitor of caspase-6 provides protection from mutant Huntingtin-induced motor and behavioral deficits. Hum Mol Genet (2015) 0.80
Integration-independent Transgenic Huntington Disease Fragment Mouse Models Reveal Distinct Phenotypes and Life Span in Vivo. J Biol Chem (2015) 0.79
Effect of methylprednisolone on the activities of caspase-3, -6, -8 and -9 in rabbits with acute spinal cord injury. Exp Ther Med (2012) 0.79
Palmitoylation of caspase-6 by HIP14 regulates its activation. Cell Death Differ (2016) 0.76
Caspase-6 Undergoes a Distinct Helix-Strand Interconversion upon Substrate Binding. J Biol Chem (2017) 0.75
An Intrabody Drug (rAAV6-INT41) Reduces the Binding of N-Terminal Huntingtin Fragment(s) to DNA to Basal Levels in PC12 Cells and Delays Cognitive Loss in the R6/2 Animal Model. J Neurodegener Dis (2016) 0.75
Sox11 Reduces Caspase-6 Cleavage and Activity. PLoS One (2015) 0.75
The P42 peptide and Peptide-based therapies for Huntington's disease. Orphanet J Rare Dis (2016) 0.75
More alive than dead: non-apoptotic roles for caspases in neuronal development, plasticity and disease. Cell Death Differ (2017) 0.75
Post-translational modifications (PTMs), identified on endogenous huntingtin, cluster within proteolytic domains between HEAT repeats. J Proteome Res (2017) 0.75
Mutations in HFE2 cause iron overload in chromosome 1q-linked juvenile hemochromatosis. Nat Genet (2003) 5.82
Huntingtin interacts with REST/NRSF to modulate the transcription of NRSE-controlled neuronal genes. Nat Genet (2003) 4.76
Cleavage at the caspase-6 site is required for neuronal dysfunction and degeneration due to mutant huntingtin. Cell (2006) 4.53
Early mitochondrial calcium defects in Huntington's disease are a direct effect of polyglutamines. Nat Neurosci (2002) 4.15
Selective striatal neuronal loss in a YAC128 mouse model of Huntington disease. Hum Mol Genet (2003) 4.07
Human and mouse proteases: a comparative genomic approach. Nat Rev Genet (2003) 4.02
Genetic variants in TPMT and COMT are associated with hearing loss in children receiving cisplatin chemotherapy. Nat Genet (2009) 3.83
Matrix metalloproteinase processing of monocyte chemoattractant proteins generates CC chemokine receptor antagonists with anti-inflammatory properties in vivo. Blood (2002) 3.58
Beta-cell ABCA1 influences insulin secretion, glucose homeostasis and response to thiazolidinedione treatment. Nat Med (2007) 3.53
Targeted inactivation of hepatic Abca1 causes profound hypoalphalipoproteinemia and kidney hypercatabolism of apoA-I. J Clin Invest (2005) 3.25
Huntingtin and huntingtin-associated protein 1 influence neuronal calcium signaling mediated by inositol-(1,4,5) triphosphate receptor type 1. Neuron (2003) 3.25
Protease degradomics: a new challenge for proteomics. Nat Rev Mol Cell Biol (2002) 3.17
Increased sensitivity to N-methyl-D-aspartate receptor-mediated excitotoxicity in a mouse model of Huntington's disease. Neuron (2002) 3.15
Proteome-derived, database-searchable peptide libraries for identifying protease cleavage sites. Nat Biotechnol (2008) 3.07
Huntingtin-interacting protein HIP14 is a palmitoyl transferase involved in palmitoylation and trafficking of multiple neuronal proteins. Neuron (2004) 3.04
Differential modulation of endotoxin responsiveness by human caspase-12 polymorphisms. Nature (2004) 2.98
Mutant frizzled-4 disrupts retinal angiogenesis in familial exudative vitreoretinopathy. Nat Genet (2002) 2.90
Mutant huntingtin binds the mitochondrial fission GTPase dynamin-related protein-1 and increases its enzymatic activity. Nat Med (2011) 2.87
Central nervous system depression of neonates breastfed by mothers receiving oxycodone for postpartum analgesia. J Pediatr (2011) 2.86
A novel pathogenic pathway of immune activation detectable before clinical onset in Huntington's disease. J Exp Med (2008) 2.78
Loss of collagenase-2 confers increased skin tumor susceptibility to male mice. Nat Genet (2003) 2.69
Balance between synaptic versus extrasynaptic NMDA receptor activity influences inclusions and neurotoxicity of mutant huntingtin. Nat Med (2009) 2.66
HIV-induced metalloproteinase processing of the chemokine stromal cell derived factor-1 causes neurodegeneration. Nat Neurosci (2003) 2.66
Lipoprotein lipase S447X: a naturally occurring gain-of-function mutation. Arterioscler Thromb Vasc Biol (2006) 2.55
Intestinal ABCA1 directly contributes to HDL biogenesis in vivo. J Clin Invest (2006) 2.55
Mutant huntingtin's effects on striatal gene expression in mice recapitulate changes observed in human Huntington's disease brain and do not differ with mutant huntingtin length or wild-type huntingtin dosage. Hum Mol Genet (2007) 2.54
Recruitment and activation of caspase-8 by the Huntingtin-interacting protein Hip-1 and a novel partner Hippi. Nat Cell Biol (2002) 2.50
Association between increased arterial-wall thickness and impairment in ABCA1-driven cholesterol efflux: an observational study. Lancet (2002) 2.39
Accurate prediction of the functional significance of single nucleotide polymorphisms and mutations in the ABCA1 gene. PLoS Genet (2005) 2.38
Complete rescue of lipoprotein lipase-deficient mice by somatic gene transfer of the naturally occurring LPLS447X beneficial mutation. Arterioscler Thromb Vasc Biol (2005) 2.36
Early increase in extrasynaptic NMDA receptor signaling and expression contributes to phenotype onset in Huntington's disease mice. Neuron (2010) 2.36
Relationship between stearoyl-CoA desaturase activity and plasma triglycerides in human and mouse hypertriglyceridemia. J Lipid Res (2002) 2.28
Mutant DNA-binding domain of HSF4 is associated with autosomal dominant lamellar and Marner cataract. Nat Genet (2002) 2.21
Variations on a gene: rare and common variants in ABCA1 and their impact on HDL cholesterol levels and atherosclerosis. Annu Rev Nutr (2006) 2.20
Palmitoylation of huntingtin by HIP14 is essential for its trafficking and function. Nat Neurosci (2006) 2.19
Beyond disgust: impaired recognition of negative emotions prior to diagnosis in Huntington's disease. Brain (2007) 2.17
IKK phosphorylates Huntingtin and targets it for degradation by the proteasome and lysosome. J Cell Biol (2009) 2.16
Disturbed Ca2+ signaling and apoptosis of medium spiny neurons in Huntington's disease. Proc Natl Acad Sci U S A (2005) 2.15
Increased ABCA1 activity protects against atherosclerosis. J Clin Invest (2002) 2.13
Intramuscular administration of AAV1-lipoprotein lipase S447X lowers triglycerides in lipoprotein lipase-deficient patients. Arterioscler Thromb Vasc Biol (2008) 2.10
Caspase cleavage of mutant huntingtin precedes neurodegeneration in Huntington's disease. J Neurosci (2002) 2.05
Matrix metalloproteinases: what do they not do? New substrates and biological roles identified by murine models and proteomics. Biochim Biophys Acta (2009) 2.04
ABCA1 mRNA and protein distribution patterns predict multiple different roles and levels of regulation. Lab Invest (2002) 2.03
Perceptions of genetic discrimination among people at risk for Huntington's disease: a cross sectional survey. BMJ (2009) 2.02
LPS responsiveness and neutrophil chemotaxis in vivo require PMN MMP-8 activity. PLoS One (2007) 2.01
Macrophage ATP-binding cassette transporter A1 overexpression inhibits atherosclerotic lesion progression in low-density lipoprotein receptor knockout mice. Arterioscler Thromb Vasc Biol (2006) 2.00
Proteomics discovery of metalloproteinase substrates in the cellular context by iTRAQ labeling reveals a diverse MMP-2 substrate degradome. Mol Cell Proteomics (2007) 1.98
Isotopic labeling of terminal amines in complex samples identifies protein N-termini and protease cleavage products. Nat Biotechnol (2010) 1.98
Spontaneous atherosclerosis in aged lipoprotein lipase-deficient mice with severe hypertriglyceridemia on a normal chow diet. Circ Res (2007) 1.94
Membrane protease proteomics: Isotope-coded affinity tag MS identification of undescribed MT1-matrix metalloproteinase substrates. Proc Natl Acad Sci U S A (2004) 1.91
Absence of behavioral abnormalities and neurodegeneration in vivo despite widespread neuronal huntingtin inclusions. Proc Natl Acad Sci U S A (2005) 1.87
When good drugs go bad. Nature (2007) 1.85
A genome scan for modifiers of age at onset in Huntington disease: The HD MAPS study. Am J Hum Genet (2003) 1.85
Cognitive dysfunction precedes neuropathology and motor abnormalities in the YAC128 mouse model of Huntington's disease. J Neurosci (2005) 1.82
Deficiency of ABCA1 impairs apolipoprotein E metabolism in brain. J Biol Chem (2004) 1.82
Inhibition of calpain cleavage of huntingtin reduces toxicity: accumulation of calpain/caspase fragments in the nucleus. J Biol Chem (2004) 1.82
Potent and selective antisense oligonucleotides targeting single-nucleotide polymorphisms in the Huntington disease gene / allele-specific silencing of mutant huntingtin. Mol Ther (2011) 1.82
HIP14, a novel ankyrin domain-containing protein, links huntingtin to intracellular trafficking and endocytosis. Hum Mol Genet (2002) 1.79
CAG expansion in the Huntington disease gene is associated with a specific and targetable predisposing haplogroup. Am J Hum Genet (2009) 1.78
Targeting tumor hypoxia: suppression of breast tumor growth and metastasis by novel carbonic anhydrase IX inhibitors. Cancer Res (2011) 1.76
The absence of ABCA1 decreases soluble ApoE levels but does not diminish amyloid deposition in two murine models of Alzheimer disease. J Biol Chem (2005) 1.74
Medicine. Whole-genome sequencing: the new standard of care? Science (2012) 1.73
Deranged neuronal calcium signaling and Huntington disease. Biochem Biophys Res Commun (2004) 1.72
Accumulation of N-terminal mutant huntingtin in mouse and monkey models implicated as a pathogenic mechanism in Huntington's disease. Hum Mol Genet (2008) 1.70
Identification of a novel gene (HSN2) causing hereditary sensory and autonomic neuropathy type II through the Study of Canadian Genetic Isolates. Am J Hum Genet (2004) 1.70
Mutation of conserved cysteines in the Ly6 domain of GPIHBP1 in familial chylomicronemia. J Lipid Res (2009) 1.67
Matrix metalloproteinase proteomics: substrates, targets, and therapy. Curr Opin Cell Biol (2009) 1.65
More codeine fatalities after tonsillectomy in North American children. Pediatrics (2012) 1.63
Pharmacogenomic prediction of anthracycline-induced cardiotoxicity in children. J Clin Oncol (2011) 1.62
A functional ABCA1 gene variant is associated with low HDL-cholesterol levels and shows evidence of positive selection in Native Americans. Hum Mol Genet (2010) 1.60
Tetrabenazine. Nat Rev Drug Discov (2009) 1.60
A novel apoA-I mutation (L178P) leads to endothelial dysfunction, increased arterial wall thickness, and premature coronary artery disease. J Am Coll Cardiol (2004) 1.58
Phosphorylation of huntingtin at Ser421 in YAC128 neurons is associated with protection of YAC128 neurons from NMDA-mediated excitotoxicity and is modulated by PP1 and PP2A. J Neurosci (2010) 1.58
Matrix metalloproteinase processing of CXCL11/I-TAC results in loss of chemoattractant activity and altered glycosaminoglycan binding. J Biol Chem (2008) 1.55
Neuronal palmitoyl acyl transferases exhibit distinct substrate specificity. FASEB J (2009) 1.55
Metadegradomics: toward in vivo quantitative degradomics of proteolytic post-translational modifications of the cancer proteome. Mol Cell Proteomics (2008) 1.55
Verbal episodic memory declines prior to diagnosis in Huntington's disease. Neuropsychologia (2007) 1.54
Automated deformation analysis in the YAC128 Huntington disease mouse model. Neuroimage (2007) 1.54