Effects of CAG repeat length, HTT protein length and protein context on cerebral metabolism measured using magnetic resonance spectroscopy in transgenic mouse models of Huntington's disease.

PubWeight™: 1.04‹?› | Rank: Top 15%

🔗 View Article (PMID 16135087)

Published in J Neurochem on August 31, 2005

Authors

Bruce G Jenkins1, Ole A Andreassen, Alpaslan Dedeoglu, Blair Leavitt, Michael Hayden, David Borchelt, Christopher A Ross, Robert J Ferrante, M Flint Beal

Author Affiliations

1: MGH-NMR Center, Department of Radiology, Massachusetts General Hospital and Harvard Medical School, Charlestown, Massachusetts 02129, USA. bgj@nmr.mgh.harvard.edu

Articles citing this

Cerebral cortex and the clinical expression of Huntington's disease: complexity and heterogeneity. Brain (2008) 2.40

Huntington's disease: can mice lead the way to treatment? Neuron (2011) 1.71

Comparison of analytical mathematical approaches for identifying key nuclear magnetic resonance spectroscopy biomarkers in the diagnosis and assessment of clinical change of diseases. J Comp Neurol (2010) 1.71

Mutant huntingtin and mitochondrial dysfunction. Trends Neurosci (2008) 1.59

Thalamic metabolism and symptom onset in preclinical Huntington's disease. Brain (2007) 1.54

Neurochemical changes in Huntington R6/2 mouse striatum detected by in vivo 1H NMR spectroscopy. J Neurochem (2007) 1.38

Cardiac dysfunction in the R6/2 mouse model of Huntington's disease. Neurobiol Dis (2006) 1.31

Brain networks in Huntington disease. J Clin Invest (2011) 1.02

Therapeutic perspectives for the treatment of Huntington's disease: treating the whole body. Histol Histopathol (2008) 0.99

Molecular characterization of skeletal muscle atrophy in the R6/2 mouse model of Huntington's disease. Am J Physiol Endocrinol Metab (2011) 0.97

Cortical metabolites as biomarkers in the R6/2 model of Huntington's disease. J Cereb Blood Flow Metab (2011) 0.91

Complexity and heterogeneity: what drives the ever-changing brain in Huntington's disease? Ann N Y Acad Sci (2008) 0.91

Homeostatic adaptations in brain energy metabolism in mouse models of Huntington disease. J Cereb Blood Flow Metab (2012) 0.86

R-flurbiprofen improves tau, but not Aß pathology in a triple transgenic model of Alzheimer's disease. Brain Res (2013) 0.84

Metabolic disturbances in diseases with neurological involvement. Aging Dis (2013) 0.84

Mouse models of polyglutamine diseases: review and data table. Part I. Mol Neurobiol (2012) 0.84

Probing the metabolic aberrations underlying mutant huntingtin toxicity in yeast and assessing their degree of preservation in humans and mice. J Proteome Res (2010) 0.81

The effects of aging, housing and ibuprofen treatment on brain neurochemistry in a triple transgene Alzheimer's disease mouse model using magnetic resonance spectroscopy and imaging. Brain Res (2014) 0.80

Spatiotemporal mapping of brain atrophy in mouse models of Huntington's disease using longitudinal in vivo magnetic resonance imaging. Neuroimage (2012) 0.79

Magnetic resonance spectroscopy of regional brain metabolite markers in FALS mice and the effects of dietary creatine supplementation. Eur J Neurosci (2009) 0.78

Preclinical (1)H-MRS neurochemical profiling in neurological and psychiatric disorders. Bioanalysis (2012) 0.78

Characterization of Behavioral, Neuropathological, Brain Metabolic and Key Molecular Changes in zQ175 Knock-In Mouse Model of Huntington's Disease. PLoS One (2016) 0.76

Imaging and spectroscopic approaches to probe brain energy metabolism dysregulation in neurodegenerative diseases. J Cereb Blood Flow Metab (2017) 0.75

Articles by these authors

Large recurrent microdeletions associated with schizophrenia. Nature (2008) 20.25

Mitochondrial dysfunction and oxidative stress in neurodegenerative diseases. Nature (2006) 15.69

A call for transparent reporting to optimize the predictive value of preclinical research. Nature (2012) 14.63

Common variants conferring risk of schizophrenia. Nature (2009) 10.37

Variant of TREM2 associated with the risk of Alzheimer's disease. N Engl J Med (2012) 10.16

APP processing and synaptic function. Neuron (2003) 8.37

A mutation in APP protects against Alzheimer's disease and age-related cognitive decline. Nature (2012) 8.10

Genetic relationship between five psychiatric disorders estimated from genome-wide SNPs. Nat Genet (2013) 8.02

Dopamine neurons derived from human ES cells efficiently engraft in animal models of Parkinson's disease. Nature (2011) 7.90

Functional engraftment of human ES cell-derived dopaminergic neurons enriched by coculture with telomerase-immortalized midbrain astrocytes. Nat Med (2006) 7.21

Parkinson's disease-associated mutations in leucine-rich repeat kinase 2 augment kinase activity. Proc Natl Acad Sci U S A (2005) 6.98

Reduced-median-network analysis of complete mitochondrial DNA coding-region sequences for the major African, Asian, and European haplogroups. Am J Hum Genet (2002) 6.02

Mitochondrial pathology and muscle and dopaminergic neuron degeneration caused by inactivation of Drosophila Pink1 is rescued by Parkin. Proc Natl Acad Sci U S A (2006) 5.78

Kinase activity of mutant LRRK2 mediates neuronal toxicity. Nat Neurosci (2006) 5.32

Pink1 regulates mitochondrial dynamics through interaction with the fission/fusion machinery. Proc Natl Acad Sci U S A (2008) 4.97

Disruption of the neurexin 1 gene is associated with schizophrenia. Hum Mol Genet (2008) 4.78

Effects of coenzyme Q10 in early Parkinson disease: evidence of slowing of the functional decline. Arch Neurol (2002) 4.60

Minocycline inhibits cytochrome c release and delays progression of amyotrophic lateral sclerosis in mice. Nature (2002) 4.21

Treatment-resistant bipolar depression: a randomized controlled trial of electroconvulsive therapy versus algorithm-based pharmacological treatment. Am J Psychiatry (2014) 3.98

Parkin mediates nonclassical, proteasomal-independent ubiquitination of synphilin-1: implications for Lewy body formation. J Neurosci (2005) 3.92

A schizophrenia-associated mutation of DISC1 perturbs cerebral cortex development. Nat Cell Biol (2005) 3.89

Parkinson's disease. Hum Mol Genet (2007) 3.84

Identification of common variants associated with human hippocampal and intracranial volumes. Nat Genet (2012) 3.73

Preparing for preventive clinical trials: the Predict-HD study. Arch Neurol (2006) 3.57

Neural subtype specification of fertilization and nuclear transfer embryonic stem cells and application in parkinsonian mice. Nat Biotechnol (2003) 3.43

Mitochondrial biology and oxidative stress in Parkinson disease pathogenesis. Nat Clin Pract Neurol (2008) 3.35

Disrupted-in-Schizophrenia-1 (DISC-1): mutant truncation prevents binding to NudE-like (NUDEL) and inhibits neurite outgrowth. Proc Natl Acad Sci U S A (2002) 3.34

Exaggerated inflammation, impaired host defense, and neuropathology in progranulin-deficient mice. J Exp Med (2009) 3.32

Mutant LRRK2(R1441G) BAC transgenic mice recapitulate cardinal features of Parkinson's disease. Nat Neurosci (2009) 3.24

Intraneuronal Alzheimer abeta42 accumulates in multivesicular bodies and is associated with synaptic pathology. Am J Pathol (2002) 3.23

p53 mediates cellular dysfunction and behavioral abnormalities in Huntington's disease. Neuron (2005) 3.13

Leucine-rich repeat kinase 2 (LRRK2) interacts with parkin, and mutant LRRK2 induces neuronal degeneration. Proc Natl Acad Sci U S A (2005) 3.00

Potential endpoints for clinical trials in premanifest and early Huntington's disease in the TRACK-HD study: analysis of 24 month observational data. Lancet Neurol (2011) 2.99

A comprehensive family-based replication study of schizophrenia genes. JAMA Psychiatry (2013) 2.93

Genetic or pharmacological iron chelation prevents MPTP-induced neurotoxicity in vivo: a novel therapy for Parkinson's disease. Neuron (2003) 2.80

Alzheimer's brains harbor somatic mtDNA control-region mutations that suppress mitochondrial transcription and replication. Proc Natl Acad Sci U S A (2004) 2.77

Amyloid beta, mitochondrial dysfunction and synaptic damage: implications for cognitive decline in aging and Alzheimer's disease. Trends Mol Med (2008) 2.74

Mutated human SOD1 causes dysfunction of oxidative phosphorylation in mitochondria of transgenic mice. J Biol Chem (2002) 2.68

Mutant huntingtin impairs axonal trafficking in mammalian neurons in vivo and in vitro. Mol Cell Biol (2004) 2.65

Inactivation of Drosophila DJ-1 leads to impairments of oxidative stress response and phosphatidylinositol 3-kinase/Akt signaling. Proc Natl Acad Sci U S A (2005) 2.58

Mitochondrial alpha-ketoglutarate dehydrogenase complex generates reactive oxygen species. J Neurosci (2004) 2.57

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

Psychopathology in patients with degenerative cerebellar diseases: a comparison to Huntington's disease. Am J Psychiatry (2002) 2.52

Identification of low-frequency variants associated with gout and serum uric acid levels. Nat Genet (2011) 2.48

Urate as a predictor of the rate of clinical decline in Parkinson disease. Arch Neurol (2009) 2.46

Neuroprotective effects of phenylbutyrate in the N171-82Q transgenic mouse model of Huntington's disease. J Biol Chem (2004) 2.43

Histone deacetylase inhibitors prevent oxidative neuronal death independent of expanded polyglutamine repeats via an Sp1-dependent pathway. Proc Natl Acad Sci U S A (2003) 2.21

Common variants at VRK2 and TCF4 conferring risk of schizophrenia. Hum Mol Genet (2011) 2.21

Beyond disgust: impaired recognition of negative emotions prior to diagnosis in Huntington's disease. Brain (2007) 2.17

Huntingtin spheroids and protofibrils as precursors in polyglutamine fibrilization. J Biol Chem (2002) 2.12

A Drosophila model for LRRK2-linked parkinsonism. Proc Natl Acad Sci U S A (2008) 2.12

Prenatal interaction of mutant DISC1 and immune activation produces adult psychopathology. Biol Psychiatry (2010) 2.11

Somatic mitochondrial DNA mutations in early Parkinson and incidental Lewy body disease. Ann Neurol (2012) 2.08

All SNPs are not created equal: genome-wide association studies reveal a consistent pattern of enrichment among functionally annotated SNPs. PLoS Genet (2013) 2.08

Lipid peroxidation in aging brain and Alzheimer's disease. Free Radic Biol Med (2002) 2.08

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

Up-regulation of NOTCH4 gene expression in bipolar disorder. Am J Psychiatry (2012) 2.04

Acute kidney injury leads to inflammation and functional changes in the brain. J Am Soc Nephrol (2008) 2.03

Sensitivity to oxidative stress in DJ-1-deficient dopamine neurons: an ES- derived cell model of primary Parkinsonism. PLoS Biol (2004) 2.02

Therapeutic effects of coenzyme Q10 and remacemide in transgenic mouse models of Huntington's disease. J Neurosci (2002) 2.01

Gene variants associated with schizophrenia in a Norwegian genome-wide study are replicated in a large European cohort. J Psychiatr Res (2010) 1.98

Neuroprotective role of Sirt1 in mammalian models of Huntington's disease through activation of multiple Sirt1 targets. Nat Med (2011) 1.97

Mitochondrial dysfunction and amyotrophic lateral sclerosis. Muscle Nerve (2006) 1.95

Chronology of behavioral symptoms and neuropathological sequela in R6/2 Huntington's disease transgenic mice. J Comp Neurol (2005) 1.95

Maternally derived microduplications at 15q11-q13: implication of imprinted genes in psychotic illness. Am J Psychiatry (2011) 1.94

Dopaminergic neuronal loss, reduced neurite complexity and autophagic abnormalities in transgenic mice expressing G2019S mutant LRRK2. PLoS One (2011) 1.93

Translational control of inducible nitric oxide synthase expression by arginine can explain the arginine paradox. Proc Natl Acad Sci U S A (2003) 1.92

Endoplasmic reticulum stress and mitochondrial cell death pathways mediate A53T mutant alpha-synuclein-induced toxicity. Hum Mol Genet (2005) 1.92

Therapeutic effects of cystamine in a murine model of Huntington's disease. J Neurosci (2002) 1.92

Triterpenoids CDDO-ethyl amide and CDDO-trifluoroethyl amide improve the behavioral phenotype and brain pathology in a transgenic mouse model of Huntington's disease. Free Radic Biol Med (2010) 1.91

Sodium phenylbutyrate prolongs survival and regulates expression of anti-apoptotic genes in transgenic amyotrophic lateral sclerosis mice. J Neurochem (2005) 1.91

Resveratrol protects against peripheral deficits in a mouse model of Huntington's disease. Exp Neurol (2010) 1.91

The ENIGMA Consortium: large-scale collaborative analyses of neuroimaging and genetic data. Brain Imaging Behav (2014) 1.90

The impact of divergence time on the nature of population structure: an example from Iceland. PLoS Genet (2009) 1.89

Transcriptional therapy with the histone deacetylase inhibitor trichostatin A ameliorates experimental autoimmune encephalomyelitis. J Neuroimmunol (2005) 1.89

Brain expressed microRNAs implicated in schizophrenia etiology. PLoS One (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

A common MECP2 haplotype associates with reduced cortical surface area in humans in two independent populations. Proc Natl Acad Sci U S A (2009) 1.84

Dense genotyping of immune-related disease regions identifies nine new risk loci for primary sclerosing cholangitis. Nat Genet (2013) 1.83

ESET/SETDB1 gene expression and histone H3 (K9) trimethylation in Huntington's disease. Proc Natl Acad Sci U S A (2006) 1.82

Impaired mitochondrial function in psychiatric disorders. Nat Rev Neurosci (2012) 1.80

Mutant superoxide dismutase 1 forms aggregates in the brain mitochondrial matrix of amyotrophic lateral sclerosis mice. J Neurosci (2005) 1.79

Ciliogenesis is regulated by a huntingtin-HAP1-PCM1 pathway and is altered in Huntington disease. J Clin Invest (2011) 1.79

The energetics of Huntington's disease. Neurochem Res (2004) 1.79

Oxidative damage in Huntington's disease pathogenesis. Antioxid Redox Signal (2006) 1.79

Striatal and white matter predictors of estimated diagnosis for Huntington disease. Brain Res Bull (2010) 1.79

"Frontal" behaviors before the diagnosis of Huntington's disease and their relationship to markers of disease progression: evidence of early lack of awareness. J Neuropsychiatry Clin Neurosci (2010) 1.76

Dietary supplementation with resveratrol reduces plaque pathology in a transgenic model of Alzheimer's disease. Neurochem Int (2008) 1.76

Transcriptional modulator H2A histone family, member Y (H2AFY) marks Huntington disease activity in man and mouse. Proc Natl Acad Sci U S A (2011) 1.76

High aggregate burden of somatic mtDNA point mutations in aging and Alzheimer's disease brain. Hum Mol Genet (2002) 1.75

Alpha-synuclein phosphorylation enhances eosinophilic cytoplasmic inclusion formation in SH-SY5Y cells. J Neurosci (2005) 1.75

Similar immune profile in bipolar disorder and schizophrenia: selective increase in soluble tumor necrosis factor receptor I and von Willebrand factor. Bipolar Disord (2009) 1.74

Cortical thickness and subcortical volumes in schizophrenia and bipolar disorder. Biol Psychiatry (2010) 1.73

Motor abnormalities in premanifest persons with Huntington's disease: the PREDICT-HD study. Mov Disord (2009) 1.73