PGC-1α, Sirtuins and PARPs in Huntington’s Disease and Other Neurodegenerative Conditions: NAD+ to Rule Them All

Niacin in the Central Nervous System: An Update of Biological Aspects and Clinical Applications

(2019) Valeria Gasperi et al.   INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES

Huntington’s disease: the coming of age

(2018) Mritunjay Pandey et al.   JOURNAL OF GENETICS

The Emergence of the Nicotinamide Riboside Kinases in the regulation of NAD+ Metabolism

(2018) Rachel S Fletcher et al.   JOURNAL OF MOLECULAR ENDOCRINOLOGY

Multiple clinical features of Huntington’s disease correlate with mutant HTT gene CAG repeat lengths and neurodegeneration

(2018) Sonia Podvin et al.   JOURNAL OF NEUROLOGY

Metabolic aspects of neuronal degeneration: From a NAD + point of view

(2018) Yo Sasaki   NEUROSCIENCE RESEARCH

Amelioration of Huntington's disease phenotypes by Beta-Lapachone is associated with increases in Sirt1 expression, CREB phosphorylation and PGC-1α deacetylation

(2018) Mijung Lee et al.   PLoS One

NAD+supplementation normalizes key Alzheimer’s features and DNA damage responses in a new AD mouse model with introduced DNA repair deficiency

(2018) Yujun Hou et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Chronic nicotinamide riboside supplementation is well-tolerated and elevates NAD+ in healthy middle-aged and older adults

(2018) Christopher R. Martens et al.   Nature Communications

Increased nuclear DNA damage precedes mitochondrial dysfunction in peripheral blood mononuclear cells from Huntington’s disease patients

(2018) Georgina Askeland et al.   Scientific Reports

The NAD+ Precursor Nicotinamide Riboside Rescues Mitochondrial Defects and Neuronal Loss in iPSC and Fly Models of Parkinson’s Disease

(2018) David C. Schöndorf et al.   Cell Reports

A transgenic minipig model of Huntington's disease shows early signs of behavioral and molecular pathologies

(2018) Georgina Askeland et al.   Disease Models & Mechanisms

Nicotinamide reverses behavioral impairments and provides neuroprotection in 3˗nitropropionic acid induced animal model ofHuntington’s disease: implication of oxidative stress˗ poly(ADP˗ ribose) polymerase pathway

(2018) Akram Sidhu et al.   METABOLIC BRAIN DISEASE

Mitochondrial alterations accompanied by oxidative stress conditions in skin fibroblasts of Huntington’s disease patients

(2018) Paulina Jędrak et al.   METABOLIC BRAIN DISEASE

A Patient-Derived Cellular Model for Huntington's Disease Reveals Phenotypes at Clinically Relevant CAG Lengths

(2018) Claudia Lin-Kar Hung et al.   MOLECULAR BIOLOGY OF THE CELL

Nuclear poly(ADP-ribose) activity is a therapeutic target in amyotrophic lateral sclerosis

(2018) L. McGurk et al.   Acta Neuropathologica Communications

An overview of Sirtuins as potential therapeutic target: Structure, function and modulators

(2018) Yijie Wang et al.   EUROPEAN JOURNAL OF MEDICINAL CHEMISTRY

Poly(ADP-ribose) drives pathologic α-synuclein neurodegeneration in Parkinson’s disease

(2018) Tae-In Kam et al.   SCIENCE

Sirtuins in Neuroendocrine Regulation and Neurological Diseases

(2018) Yuki Fujita et al.   Frontiers in Neuroscience

Nicotinamide riboside, a form of vitamin B3, protects against excitotoxicity-induced axonal degeneration

(2017) Pauline Vaur et al.   FASEB JOURNAL

Base excision repair of oxidative DNA damage from mechanism to disease

(2017) Bret D Freudenthal   Frontiers in Bioscience-Landmark

DNA Polymerase Beta Participates in Mitochondrial DNA Repair

(2017) P. Sykora et al.   MOLECULAR AND CELLULAR BIOLOGY

Combination of NAD+ and NADPH Offers Greater Neuroprotection in Ischemic Stroke Models by Relieving Metabolic Stress

(2017) Qiao Huang et al.   MOLECULAR NEUROBIOLOGY

Selective Sparing of Striatal Interneurons after Poly (ADP-Ribose) Polymerase 1 Inhibition in the R6/2 Mouse Model of Huntington’s Disease

(2017) Emanuela Paldino et al.   Frontiers in Neuroanatomy

DNA polymerase β decrement triggers death of olfactory bulb cells and impairs olfaction in a mouse model of Alzheimer's disease

(2016) Magdalena Misiak et al.   AGING CELL

NAD + Replenishment Improves Lifespan and Healthspan in Ataxia Telangiectasia Models via Mitophagy and DNA Repair

(2016) Evandro Fei Fang et al.   Cell Metabolism

Mitochondrial SIRT3 Mediates Adaptive Responses of Neurons to Exercise and Metabolic and Excitatory Challenges

(2016) Aiwu Cheng et al.   Cell Metabolism

Exosomes from adipose-derived stem cells ameliorate phenotype of Huntington's diseasein vitromodel

(2016) Mijung Lee et al.   EUROPEAN JOURNAL OF NEUROSCIENCE

Safety assessment of nicotinamide riboside, a form of vitamin B3

(2016) DB Conze et al.   HUMAN & EXPERIMENTAL TOXICOLOGY

Huntingtin N17 domain is a reactive oxygen species sensor regulating huntingtin phosphorylation and localization

(2016) Laura F. DiGiovanni et al.   HUMAN MOLECULAR GENETICS

Bioenergetic Impairment in Animal and Cellular Models of Alzheimer’s Disease: PARP-1 Inhibition Rescues Metabolic Dysfunctions

(2016) Sara Martire et al.   JOURNAL OF ALZHEIMERS DISEASE

Enhancing NAD+Salvage Pathway Reverts the Toxicity of Primary Astrocytes Expressing Amyotrophic Lateral Sclerosis-linked Mutant Superoxide Dismutase 1 (SOD1)

(2016) Benjamin A. Harlan et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Comparative Mitochondrial-Based Protective Effects of Resveratrol and Nicotinamide in Huntington’s Disease Models

(2016) Luana Naia et al.   MOLECULAR NEUROBIOLOGY

XRCC1 mutation is associated with PARP1 hyperactivation and cerebellar ataxia

(2016) Nicolas C. Hoch et al.   NATURE

NAD+ repletion improves mitochondrial and stem cell function and enhances life span in mice

(2016) H. Zhang et al.   SCIENCE

Parp mutations protect against mitochondrial dysfunction and neurodegeneration in a PARKIN model of Parkinson’s disease

(2016) S Lehmann et al.   Cell Death & Disease

NRK1 controls nicotinamide mononucleotide and nicotinamide riboside metabolism in mammalian cells

(2016) Joanna Ratajczak et al.   Nature Communications

Huntingtin’s spherical solenoid structure enables polyglutamine tract-dependent modulation of its structure and function

(2016) Ravi Vijayvargia et al.   eLife

Regulation of the mPTP by SIRT3-mediated deacetylation of CypD at lysine 166 suppresses age-related cardiac hypertrophy

(2016) Angela V. Hafner et al.   Aging-US

SIRT1 Activity Is Linked to Its Brain Region-Specific Phosphorylation and Is Impaired in Huntington’s Disease Mice

(2016) Raffaella Tulino et al.   PLoS One

SIRT3 deficiency impairs mitochondrial and contractile function in the heart

(2015) Christoph Koentges et al.   BASIC RESEARCH IN CARDIOLOGY

Safety, pharmacokinetics, pharmacogenomics and QT concentration−effect modelling of the SirT1 inhibitor selisistat in healthy volunteers

(2015) Goran Westerberg et al.   BRITISH JOURNAL OF CLINICAL PHARMACOLOGY

An exploratory double-blind, randomized clinical trial with selisistat, a SirT1 inhibitor, in patients with Huntington's disease

(2015) Sigurd D. Süssmuth et al.   BRITISH JOURNAL OF CLINICAL PHARMACOLOGY

Huntingtin functions as a scaffold for selective macroautophagy

(2015) Yan-Ning Rui et al.   NATURE CELL BIOLOGY

PARP-1 Inhibition Is Neuroprotective in the R6/2 Mouse Model of Huntington’s Disease

(2015) Antonella Cardinale et al.   PLoS One

OXPHOS-Mediated Induction of NAD+ Promotes Complete Oxidation of Fatty Acids and Interdicts Non-Alcoholic Fatty Liver Disease

(2015) Thomas E. Akie et al.   PLoS One

DNA polymerase β deficiency leads to neurodegeneration and exacerbates Alzheimer disease phenotypes

(2015) Peter Sykora et al.   NUCLEIC ACIDS RESEARCH

Mouse SIRT3 Attenuates Hypertrophy-Related Lipid Accumulation in the Heart through the Deacetylation of LCAD

(2015) Tongshuai Chen et al.   PLoS One

Differentiation-Associated Downregulation of Poly(ADP-Ribose) Polymerase-1 Expression in Myoblasts Serves to Increase Their Resistance to Oxidative Stress

(2015) Gábor Oláh et al.   PLoS One

A single nucleotide polymorphism in the coding region of PGC-1α is a male-specific modifier of Huntington disease age-at-onset in a large European cohort

(2014) Patrick Weydt et al.   BMC Neurology

Activation of SIRT3 by the NAD+ Precursor Nicotinamide Riboside Protects from Noise-Induced Hearing Loss

(2014) Kevin D. Brown et al.   Cell Metabolism

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

Prospects for neuroprotective therapies in prodromal Huntington's disease

(2014) Abhishek Chandra et al.   MOVEMENT DISORDERS

Role of oxidative DNA damage in mitochondrial dysfunction and Huntington’s disease pathogenesis

(2013) Sylvette Ayala-Peña   FREE RADICAL BIOLOGY AND MEDICINE

Crosstalk between poly(ADP-ribose) polymerase and sirtuin enzymes

(2013) Carles Cantó et al.   MOLECULAR ASPECTS OF MEDICINE

Nicotinamide riboside restores cognition through an upregulation of proliferator-activated receptor-γ coactivator 1α regulated β-secretase 1 degradation and mitochondrial gene expression in Alzheimer's mouse models

(2013) Bing Gong et al.   NEUROBIOLOGY OF AGING

Genetic models of PGC-1 and glucose metabolism and homeostasis

(2013) Glenn C. Rowe et al.   REVIEWS IN ENDOCRINE & METABOLIC DISORDERS

The Nucleotide Sequence, DNA Damage Location, and Protein Stoichiometry Influence the Base Excision Repair Outcome at CAG/CTG Repeats

(2012) Agathi-Vasiliki Goula et al.   BIOCHEMISTRY

The NAD+ Precursor Nicotinamide Riboside Enhances Oxidative Metabolism and Protects against High-Fat Diet-Induced Obesity

(2012) Carles Cantó et al.   Cell Metabolism

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

Integration of  -Catenin, Sirtuin, and FOXO Signaling Protects from Mutant Huntingtin Toxicity

(2012) J. A. Parker et al.   JOURNAL OF NEUROSCIENCE

Integrin-Dependent Akt1 Activation Regulates PGC-1 Expression and Fatty Acid Oxidation

(2012) Craig C. Beeson et al.   JOURNAL OF VASCULAR RESEARCH

New insights into the molecular and cellular functions of poly(ADP-ribose) and PARPs

(2012) Bryan A. Gibson et al.   NATURE REVIEWS MOLECULAR CELL BIOLOGY

PGC-1  Rescues Huntington's Disease Proteotoxicity by Preventing Oxidative Stress and Promoting TFEB Function

(2012) T. Tsunemi et al.   Science Translational Medicine

Involvement of PGC-1α in the formation and maintenance of neuronal dendritic spines

(2012) Aiwu Cheng et al.   Nature Communications

Cellular bioenergetics is regulated by PARP1 under resting conditions and during oxidative stress

(2011) Katalin Módis et al.   BIOCHEMICAL PHARMACOLOGY

PARP-2 Regulates SIRT1 Expression and Whole-Body Energy Expenditure

(2011) Péter Bai et al.   Cell Metabolism

PARP-1 Inhibition Increases Mitochondrial Metabolism through SIRT1 Activation

(2011) Péter Bai et al.   Cell Metabolism

Tumour suppressor SIRT3 deacetylates and activates manganese superoxide dismutase to scavenge ROS

(2011) Yaohui Chen et al.   EMBO REPORTS

Mitogen- and stress-activated protein kinase 1-induced neuroprotection in Huntington's disease: role on chromatin remodeling at the PGC-1-alpha promoter

(2011) Elodie Martin et al.   HUMAN MOLECULAR GENETICS

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

In Vitroandin VivoAggregation of a Fragment of Huntingtin Protein Directly Causes Free Radical Production

(2011) Sarah Hands et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Peroxisome-Proliferator-Activated Receptor Gamma Coactivator 1   Contributes to Dysmyelination in Experimental Models of Huntington's Disease

(2011) Z. Xiang et al.   JOURNAL OF NEUROSCIENCE

SIRT3 Deficiency and Mitochondrial Protein Hyperacetylation Accelerate the Development of the Metabolic Syndrome

(2011) Matthew D. Hirschey et al.   MOLECULAR CELL

PGC-1alpha downstream transcription factors NRF-1 and TFAM are genetic modifiers of Huntington disease

(2011) Elahe Taherzadeh-Fard et al.   Molecular Neurodegeneration

Localization of sequence variations in PGC-1α influence their modifying effect in Huntington disease

(2011) Hong Van B Che et al.   Molecular Neurodegeneration

Pharmacological Effects of Exogenous NAD on Mitochondrial Bioenergetics, DNA Repair, and Apoptosis

(2011) M. Pittelli et al.   MOLECULAR PHARMACOLOGY

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

Neuronal Sirt3 Protects against Excitotoxic Injury in Mouse Cortical Neuron Culture

(2011) Sun Hee Kim et al.   PLoS One

Brain-Derived Neurotrophic Factor in Patients with Huntington's Disease

(2011) Chiara Zuccato et al.   PLoS One

Sirtuin-3 (Sirt3) regulates skeletal muscle metabolism and insulin signaling via altered mitochondrial oxidation and reactive oxygen species production

(2011) E. Jing et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Treatment of Parkinson's disease with NADH

(2010) N. Dizdar et al.   ACTA NEUROLOGICA SCANDINAVICA

Mitochondrial and Nuclear Cross Talk in Cell Death

(2010) Shaida A. Andrabi et al.   Annals of the New York Academy of Sciences

Sirt3 Mediates Reduction of Oxidative Damage and Prevention of Age-Related Hearing Loss under Caloric Restriction

(2010) Shinichi Someya et al.   CELL

Calorie Restriction Reduces Oxidative Stress by SIRT3-Mediated SOD2 Activation

(2010) Xiaolei Qiu et al.   Cell Metabolism

Cdc2-like Kinase 2 Is an Insulin-Regulated Suppressor of Hepatic Gluconeogenesis

(2010) Joseph T. Rodgers et al.   Cell Metabolism

Resveratrol protects against peripheral deficits in a mouse model of Huntington's disease

(2010) Daniel J. Ho et al.   EXPERIMENTAL NEUROLOGY

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

(2010) J. Kim 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

Sirt3-Mediated Deacetylation of Evolutionarily Conserved Lysine 122 Regulates MnSOD Activity in Response to Stress

(2010) Randa Tao et al.   MOLECULAR CELL

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

Sirtuin 3, a New Target of PGC-1α, Plays an Important Role in the Suppression of ROS and Mitochondrial Biogenesis

(2010) Xingxing Kong et al.   PLoS One

Regulation of Succinate Dehydrogenase Activity by SIRT3 in Mammalian Mitochondria

(2009) Huseyin Cimen et al.   BIOCHEMISTRY

Cause and consequence: Mitochondrial dysfunction initiates and propagates neuronal dysfunction, neuronal death and behavioral abnormalities in age-associated neurodegenerative diseases

(2009) Gary E. Gibson et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE

Acetylation Targets Mutant Huntingtin to Autophagosomes for Degradation

(2009) Hyunkyung Jeong et al.   CELL

Somatic expansion of the Huntington's disease CAG repeat in the brain is associated with an earlier age of disease onset

(2009) Meera Swami et al.   HUMAN MOLECULAR GENETICS

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

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

PGC-1α and PGC-1β Regulate Mitochondrial Density in Neurons

(2009) Przemyslaw Wareski et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Coordination between Polymerase β and FEN1 Can Modulate CAG Repeat Expansion

(2009) Yuan Liu et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

CTG/CAG Repeat Instability Is Modulated by the Levels of Human DNA Ligase I and Its Interaction with Proliferating Cell Nuclear Antigen

(2009) Arturo López Castel et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Balance between synaptic versus extrasynaptic NMDA receptor activity influences inclusions and neurotoxicity of mutant huntingtin

(2009) Shu-ichi Okamoto et al.   NATURE MEDICINE

Serines 13 and 16 Are Critical Determinants of Full-Length Human Mutant Huntingtin Induced Disease Pathogenesis in HD Mice

(2009) Xiaofeng Gu et al.   NEURON

Stoichiometry of Base Excision Repair Proteins Correlates with Increased Somatic CAG Instability in Striatum over Cerebellum in Huntington's Disease Transgenic Mice

(2009) Agathi-Vassiliki Goula et al.   PLoS Genetics

Synergistic activation of the human MnSOD promoter by DJ-1 and PGC-1α: regulation by SUMOylation and oxidation

(2008) Nan Zhong et al.   HUMAN MOLECULAR GENETICS

Inhibition of specific HDACs and sirtuins suppresses pathogenesis in a Drosophila model of Huntington’s disease

(2008) Judit Pallos et al.   HUMAN MOLECULAR GENETICS

Nicotinamide Restores Cognition in Alzheimer's Disease Transgenic Mice via a Mechanism Involving Sirtuin Inhibition and Selective Reduction of Thr231-Phosphotau

(2008) K. N. Green et al.   JOURNAL OF NEUROSCIENCE

Intergenerational and striatal CAG repeat instability in Huntington's disease knock-in mice involve different DNA repair genes

(2008) Ella Dragileva et al.   NEUROBIOLOGY OF DISEASE

Modulation of SIRT1 expression in different neurodegenerative models and human pathologies

(2008) M. Pallàs et al.   NEUROSCIENCE

Cellular NAD Replenishment Confers Marked Neuroprotection Against Ischemic Cell Death

(2008) Suping Wang et al.   STROKE