Metabolism in HD: Still a relevant mechanism?

A potent and selective Sirtuin 1 inhibitor alleviates pathology in multiple animal and cell models of Huntington's disease

(2014) Marianne R. Smith et al.   HUMAN MOLECULAR GENETICS

PRECREST: A phase II prevention and biomarker trial of creatine in at-risk Huntington disease

(2014) H. D. Rosas et al.   NEUROLOGY

Neuroprotective effects of PPAR-γ agonist rosiglitazone in N171-82Q mouse model of Huntington's disease

(2013) Jing Jin et al.   JOURNAL OF NEUROCHEMISTRY

Evidence for a Common Mechanism of SIRT1 Regulation by Allosteric Activators

(2013) B. P. Hubbard et al.   SCIENCE

Sirtuins: from metabolic regulation to brain aging

(2013) Wenzhen Duan   Frontiers in Aging Neuroscience

Mitochondrial DNA damage Is associated with reduced mitochondrial bioenergetics in Huntington's disease

(2012) Almas Siddiqui et al.   FREE RADICAL BIOLOGY AND MEDICINE

A greatly extended PPARGC1A genomic locus encodes several new brain-specific isoforms and influences Huntington disease age of onset†

(2012) Selma M. Soyal et al.   HUMAN MOLECULAR GENETICS

trans-(−)-ϵ-Viniferin Increases Mitochondrial Sirtuin 3 (SIRT3), Activates AMP-activated Protein Kinase (AMPK), and Protects Cells in Models of Huntington Disease

(2012) Jinrong Fu et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

The Sirtuin 2 Inhibitor AK-7 Is Neuroprotective in Huntington’s Disease Mouse Models

(2012) Vanita Chopra et al.   Cell Reports

Small-Molecule Allosteric Activators of Sirtuins

(2011) David A. Sinclair et al.   Annual Review of Pharmacology and Toxicology

Pharmacologic activation of mitochondrial biogenesis exerts widespread beneficial effects in a transgenic mouse model of Huntington's disease

(2011) Ashu Johri et al.   HUMAN MOLECULAR GENETICS

Mutant huntingtin's interaction with mitochondrial protein Drp1 impairs mitochondrial biogenesis and causes defective axonal transport and synaptic degeneration in Huntington's disease

(2011) U. P. Shirendeb et al.   HUMAN MOLECULAR GENETICS

Early Alterations of Brain Cellular Energy Homeostasis in Huntington Disease Models

(2011) Fanny Mochel et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Nuclear translocation of AMPK-α1 potentiates striatal neurodegeneration in Huntington’s disease

(2011) Tz-Chuen Ju et al.   JOURNAL OF CELL BIOLOGY

Dysregulated brain creatine kinase is associated with hearing impairment in mouse models of Huntington disease

(2011) Yow-Sien Lin et al.   JOURNAL OF CLINICAL INVESTIGATION

Evidence for behavioral benefits of early dietary supplementation with CoEnzymeQ10 in a slowly progressing mouse model of Huntington's disease

(2011) Miriam A. Hickey et al.   MOLECULAR AND CELLULAR NEUROSCIENCE

Neuroprotective role of Sirt1 in mammalian models of Huntington's disease through activation of multiple Sirt1 targets

(2011) Mali Jiang et al.   NATURE MEDICINE

Sirt1 mediates neuroprotection from mutant huntingtin by activation of the TORC1 and CREB transcriptional pathway

(2011) Hyunkyung Jeong et al.   NATURE MEDICINE

Mutant huntingtin binds the mitochondrial fission GTPase dynamin-related protein-1 and increases its enzymatic activity

(2011) Wenjun Song et al.   NATURE MEDICINE

Protein oxidation in Huntington disease affects energy production and vitamin B6 metabolism

(2010) Ma Alba Sorolla et al.   FREE RADICAL BIOLOGY AND MEDICINE

Mitochondrial loss, dysfunction and altered dynamics in Huntington's disease

(2010) J. Kim et al.   HUMAN MOLECULAR GENETICS

Modulation of energy deficiency in Huntington's disease via activation of the peroxisome proliferator-activated receptor gamma

(2010) Ming-Chang Chiang et al.   HUMAN MOLECULAR GENETICS

Impairment of PGC-1alpha expression, neuropathology and hepatic steatosis in a transgenic mouse model of Huntington's disease following chronic energy deprivation

(2010) R. K. Chaturvedi et al.   HUMAN MOLECULAR GENETICS

Mitochondrial bioenergetics and dynamics in Huntington’s disease: tripartite synapses and selective striatal degeneration

(2010) Jorge M. A. Oliveira   JOURNAL OF BIOENERGETICS AND BIOMEMBRANES

SIRT2 regulates NF- B-dependent gene expression through deacetylation of p65 Lys310

(2010) K. M. Rothgiesser et al.   JOURNAL OF CELL SCIENCE

Safety and tolerability of high-dosage coenzyme Q10 in Huntington's disease and healthy subjects

(2010)   MOVEMENT DISORDERS

Nicotinamide improves motor deficits and upregulates PGC-1α and BDNF gene expression in a mouse model of Huntington's disease

(2010) Tyisha Hathorn et al.   NEUROBIOLOGY OF DISEASE

Impaired Brain Creatine Kinase Activity in Huntington’s Disease

(2010) S.F. Zhang et al.   Neurodegenerative Diseases

Comprehensive Behavioral Testing in the R6/2 Mouse Model of Huntington's Disease Shows No Benefit from CoQ10 or Minocycline

(2010) Liliana B. Menalled et al.   PLoS One

Mitochondrial calcium function and dysfunction in the central nervous system

(2009) David G. Nicholls   BIOCHIMICA ET BIOPHYSICA ACTA-BIOENERGETICS

Impaired mitochondrial trafficking in Huntington's disease

(2009) Xiao-Jiang Li et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE

Mitochondria in Huntington's disease

(2009) Maria Damiano et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE

Impaired PGC-1α function in muscle in Huntington's disease

(2009) Rajnish K. Chaturvedi et al.   HUMAN MOLECULAR GENETICS

Mitochondrial dynamics-fusion, fission, movement, and mitophagy-in neurodegenerative diseases

(2009) H. Chen et al.   HUMAN MOLECULAR GENETICS

Combination therapy with Coenzyme Q10and creatine produces additive neuroprotective effects in models of Parkinson’s and Huntington’s Diseases

(2009) Lichuan Yang et al.   JOURNAL OF NEUROCHEMISTRY

AMPK in Health and Disease

(2009) Gregory R. Steinberg et al.   PHYSIOLOGICAL REVIEWS

Mitochondrial fusion, fission and autophagy as a quality control axis: The bioenergetic view

(2008) Gilad Twig et al.   BIOCHIMICA ET BIOPHYSICA ACTA-BIOENERGETICS

Functions and effects of creatine in the central nervous system

(2008) Robert H. Andres et al.   BRAIN RESEARCH BULLETIN

Proteomic and oxidative stress analysis in human brain samples of Huntington disease

(2008) M Alba Sorolla et al.   FREE RADICAL BIOLOGY AND MEDICINE

Rosiglitazone Treatment Prevents Mitochondrial Dysfunction in Mutant Huntingtin-expressing Cells

(2008) Rodrigo A. Quintanilla et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

N-Terminal Mutant Huntingtin Associates with Mitochondria and Impairs Mitochondrial Trafficking

(2008) A. L. Orr et al.   JOURNAL OF NEUROSCIENCE

Cyclosporin A corrects mitochondrial dysfunction and muscle apoptosis in patients with collagen VI myopathies

(2008) Luciano Merlini et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA