Published in Ann N Y Acad Sci on December 01, 2008
Cargo recognition failure is responsible for inefficient autophagy in Huntington's disease. Nat Neurosci (2010) 4.22
Altered white matter microstructure in the corpus callosum in Huntington's disease: implications for cortical "disconnection". Neuroimage (2009) 1.72
Mitochondrial loss, dysfunction and altered dynamics in Huntington's disease. Hum Mol Genet (2010) 1.62
Energy deficit in Huntington disease: why it matters. J Clin Invest (2011) 1.51
Mitochondrial structural and functional dynamics in Huntington's disease. Brain Res Rev (2009) 1.37
Protein misfolding disorders and macroautophagy. Curr Opin Cell Biol (2010) 1.36
PGC-1alpha in aging and anti-aging interventions. Biochim Biophys Acta (2009) 1.35
Cause and consequence: mitochondrial dysfunction initiates and propagates neuronal dysfunction, neuronal death and behavioral abnormalities in age-associated neurodegenerative diseases. Biochim Biophys Acta (2009) 1.33
Recent advances in our understanding of neurodegeneration. J Neural Transm (Vienna) (2009) 1.27
Inhibition of transglutaminase 2 mitigates transcriptional dysregulation in models of Huntington disease. EMBO Mol Med (2010) 1.27
Differential vulnerability of neurons in Huntington's disease: the role of cell type-specific features. J Neurochem (2010) 1.25
Redox proteomics in selected neurodegenerative disorders: from its infancy to future applications. Antioxid Redox Signal (2012) 1.24
A role of mitochondrial complex II defects in genetic models of Huntington's disease expressing N-terminal fragments of mutant huntingtin. Hum Mol Genet (2013) 1.13
Role of mitochondrial dysfunction in the pathogenesis of Huntington's disease. Brain Res Bull (2009) 1.07
Mitochondrial medicine for neurodegenerative diseases. Int J Biochem Cell Biol (2010) 0.93
Meclizine is neuroprotective in models of Huntington's disease. Hum Mol Genet (2010) 0.92
Mitochondrial calcium uptake capacity as a therapeutic target in the R6/2 mouse model of Huntington's disease. Hum Mol Genet (2010) 0.92
HIF prolyl hydroxylase inhibitors prevent neuronal death induced by mitochondrial toxins: therapeutic implications for Huntington's disease and Alzheimer's disease. Antioxid Redox Signal (2010) 0.90
Dysregulation of mitochondrial calcium signaling and superoxide flashes cause mitochondrial genomic DNA damage in Huntington disease. J Biol Chem (2012) 0.90
Tractography of the corpus callosum in Huntington's disease. PLoS One (2013) 0.86
Early deficits in glycolysis are specific to striatal neurons from a rat model of huntington disease. PLoS One (2013) 0.84
Deep white matter in Huntington's disease. PLoS One (2014) 0.83
Mitochondrial bioenergetics and dynamics in Huntington's disease: tripartite synapses and selective striatal degeneration. J Bioenerg Biomembr (2010) 0.83
Trehalose reverses cell malfunction in fibroblasts from normal and Huntington's disease patients caused by proteosome inhibition. PLoS One (2014) 0.83
Dysfunctional mitochondrial respiration in the striatum of the Huntington's disease transgenic R6/2 mouse model. PLoS Curr (2013) 0.83
Mutant Huntingtin induces activation of the Bcl-2/adenovirus E1B 19-kDa interacting protein (BNip3). Cell Death Dis (2010) 0.82
Development of an ELISA assay for the quantification of soluble huntingtin in human blood cells. BMC Biochem (2013) 0.82
TRiC subunits enhance BDNF axonal transport and rescue striatal atrophy in Huntington's disease. Proc Natl Acad Sci U S A (2016) 0.81
Huntingtin protein is essential for mitochondrial metabolism, bioenergetics and structure in murine embryonic stem cells. Dev Biol (2014) 0.80
Nucleic Acid-Based Therapy Approaches for Huntington's Disease. Neurol Res Int (2012) 0.80
Targeting sirtuin-1 in Huntington's disease: rationale and current status. CNS Drugs (2013) 0.79
QEEG Measures in Huntington's Disease: A Pilot Study. PLoS Curr (2010) 0.78
Dysregulation of system xc(-) expression induced by mutant huntingtin in a striatal neuronal cell line and in R6/2 mice. Neurochem Int (2014) 0.77
The plasma membrane redox enzyme NQO1 sustains cellular energetics and protects human neuroblastoma cells against metabolic and proteotoxic stress. Age (Dordr) (2011) 0.77
Old Things New View: Ascorbic Acid Protects the Brain in Neurodegenerative Disorders. Int J Mol Sci (2015) 0.76
The calpain-suppressing effects of olesoxime in Huntington's disease. Rare Dis (2016) 0.76
Mitochondria-Targeted Antioxidant SS-31 is a Potential Novel Ophthalmic Medication for Neuroprotection in Glaucoma. Med Hypothesis Discov Innov Ophthalmol (2015) 0.75
No evidence of impaired gastric emptying in early Huntington's Disease. PLoS Curr (2011) 0.75
Enhanced neuronal glucose transporter expression reveals metabolic choice in a HD Drosophila model. PLoS One (2015) 0.75
The Association of VDAC with Cell Viability of PC12 Model of Huntington's Disease. Front Oncol (2016) 0.75
Cytochrome b5 reductase, a plasma membrane redox enzyme, protects neuronal cells against metabolic and oxidative stress through maintaining redox state and bioenergetics. Age (Dordr) (2015) 0.75
Fast-to-Slow Transition of Skeletal Muscle Contractile Function and Corresponding Changes in Myosin Heavy and Light Chain Formation in the R6/2 Mouse Model of Huntington's Disease. PLoS One (2016) 0.75
The phasor-FLIM fingerprints reveal shifts from OXPHOS to enhanced glycolysis in Huntington Disease. Sci Rep (2016) 0.75