PGC-1s in the Spotlight with Parkinson’s Disease

PARIS induced defects in mitochondrial biogenesis drive dopamine neuron loss under conditions of parkin or PINK1 deficiency

(2020) Sheila K. Pirooznia et al.   Molecular Neurodegeneration

Fibroblast mitochondria in idiopathic Parkinson’s disease display morphological changes and enhanced resistance to depolarization

(2020) P. M. A. Antony et al.   Scientific Reports

Hypermethylation of the Gene Coding for PGC-1α in Peripheral Blood Leukocytes of Patients With Parkinson’s Disease

(2020) Xiaodong Yang et al.   Frontiers in Neuroscience

Genetics of Parkinson's disease: An introspection of its journey towards precision medicine

(2020) Sara Bandres-Ciga et al.   NEUROBIOLOGY OF DISEASE

Cerebral Mitochondrial Function and Cognitive Performance during Aging: A Longitudinal Study in NMRI Mice

(2020) Martina Reutzel et al.   Oxidative Medicine and Cellular Longevity

Defects in Mitochondrial Biogenesis Drive Mitochondrial Alterations in PARKIN-Deficient Human Dopamine Neurons

(2020) Manoj Kumar et al.   Stem Cell Reports

Global burden of 369 diseases and injuries in 204 countries and territories, 1990–2019: a systematic analysis for the Global Burden of Disease Study 2019

(2020) Theo Vos et al.   LANCET

Investigation of the effect of PGC1A gene therapy at advanced stages of Alzheimer’s disease in an animal model of amyloid pathology

(2020) Bibiana Castagna Mota et al.   Alzheimers & Dementia

Mitochondrial DNA copy number in human disease: the more the better?

(2020) Roberta Filograna et al.   FEBS LETTERS

Mitochondrial Dysfunction and Mitophagy in Parkinson’s Disease: From Mechanism to Therapy

(2020) Ana Belen Malpartida et al.   TRENDS IN BIOCHEMICAL SCIENCES

Mitochondrial Dysfunction in Parkinson’s Disease: Focus on Mitochondrial DNA

(2020) Olga Buneeva et al.   Biomedicines

LRRK2 impairs PINK1/Parkin-dependent mitophagy via its kinase activity: pathologic insights into Parkinson’s disease

(2019) Fiona Bonello et al.   HUMAN MOLECULAR GENETICS

Increased Levels of cAMP by the Calcium-Dependent Activation of Soluble Adenylyl Cyclase in Parkin-Mutant Fibroblasts

(2019) Paola Tanzarella et al.   Cells

Pathogenic alpha-synuclein aggregates preferentially bind to mitochondria and affect cellular respiration

(2019) Xinhe Wang et al.   Acta Neuropathologica Communications

LRRK2 mutations impair depolarization-induced mitophagy through inhibition of mitochondrial accumulation of RAB10

(2019) Fieke Wauters et al.   Autophagy

Role of DJ-1 in the mechanism of pathogenesis of Parkinson's disease

(2019) Ludmila P. Dolgacheva et al.   JOURNAL OF BIOENERGETICS AND BIOMEMBRANES

Serotonin regulates mitochondrial biogenesis and function in rodent cortical neurons via the 5-HT2A receptor and SIRT1–PGC-1α axis

(2019) Sashaina E. Fanibunda et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Searching for Potential Lipid Biomarkers of Parkinson’s Disease in Parkin-Mutant Human Skin Fibroblasts by HILIC-ESI-MS/MS: Preliminary Findings

(2019) Calvano et al.   INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES

Lewy pathology in Parkinson’s disease consists of crowded organelles and lipid membranes

(2019) Sarah H. Shahmoradian et al.   NATURE NEUROSCIENCE

Parkinson’s disease protein DJ-1 regulates ATP synthase protein components to increase neuronal process outgrowth

(2019) Rongmin Chen et al.   Cell Death & Disease

Cell models of lipid-rich α-synuclein aggregation validate known modifiers of α-synuclein biology and identify stearoyl-CoA desaturase

(2019) Thibaut Imberdis et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Role of Oleic Acid in the Gut-Liver Axis: From Diet to the Regulation of Its Synthesis via Stearoyl-CoA Desaturase 1 (SCD1)

(2019) Elena Piccinin et al.   Nutrients

The genetics of Parkinson disease

(2018) Hao Deng et al.   AGEING RESEARCH REVIEWS

Basal Mitophagy Occurs Independently of PINK1 in Mouse Tissues of High Metabolic Demand

(2018) Thomas G. McWilliams et al.   Cell Metabolism

Mitochondrial Dysfunction in Parkinson’s Disease: New Mechanistic Insights and Therapeutic Perspectives

(2018) Jin-Sung Park et al.   Current Neurology and Neuroscience Reports

Hepatic peroxisome proliferator-activated receptor γ coactivator 1β drives mitochondrial and anabolic signatures that contribute to hepatocellular carcinoma progression in mice

(2018) Elena Piccinin et al.   HEPATOLOGY

Basal mitophagy is widespread inDrosophilabut minimally affected by loss of Pink1 or parkin

(2018) Juliette J. Lee et al.   JOURNAL OF CELL BIOLOGY

Mechanism of parkin activation by PINK1

(2018) Christina Gladkova et al.   NATURE

Building and decoding ubiquitin chains for mitophagy

(2018) J. Wade Harper et al.   NATURE REVIEWS MOLECULAR CELL BIOLOGY

Expression of the gene coading for PGC-1α in peripheral blood leukocytes and related gene variants in patients with Parkinson's disease

(2018) Xiao-Dong Yang et al.   PARKINSONISM & RELATED DISORDERS

Distinct Mechanisms of Pathogenic DJ-1 Mutations in Mitochondrial Quality Control

(2018) Daniela Strobbe et al.   Frontiers in Molecular Neuroscience

New insights into the complex role of mitochondria in Parkinson’s disease

(2018) Anne Grünewald et al.   PROGRESS IN NEUROBIOLOGY

Lipidomic Analysis of α-Synuclein Neurotoxicity Identifies Stearoyl CoA Desaturase as a Target for Parkinson Treatment

(2018) Saranna Fanning et al.   MOLECULAR CELL

Metabolic aspects in NAFLD, NASH and hepatocellular carcinoma: the role of PGC1 coactivators

(2018) Elena Piccinin et al.   Nature Reviews Gastroenterology & Hepatology

The PPARGC1A locus and CNS-specific PGC-1α isoforms are associated with Parkinson's Disease

(2018) Selma M. Soyal et al.   NEUROBIOLOGY OF DISEASE

Inhibiting Stearoyl-CoA Desaturase Ameliorates α-Synuclein Cytotoxicity

(2018) Benjamin M. Vincent et al.   Cell Reports

Parkin functionally interacts with PGC-1α to preserve mitochondria and protect dopaminergic neurons

(2017) Lu Zheng et al.   HUMAN MOLECULAR GENETICS

Lipid profiling of parkin -mutant human skin fibroblasts

(2017) Simona Lobasso et al.   JOURNAL OF CELLULAR PHYSIOLOGY

Lack of Parkin anticipates the phenotype and affects mitochondrial morphology and mtDNA levels in a mouse model of Parkinson's Disease

(2017) Milena Pinto et al.   JOURNAL OF NEUROSCIENCE

Genetic or pharmacological activation of the Drosophila PGC-1α ortholog spargel rescues the disease phenotypes of genetic models of Parkinson's disease

(2017) Chee-Hoe Ng et al.   NEUROBIOLOGY OF AGING

Recent findings on the physiological function of DJ-1: Beyond Parkinson's disease

(2017) Alice Biosa et al.   NEUROBIOLOGY OF DISEASE

Loss of Parkinson’s disease-associated protein CHCHD2 affects mitochondrial crista structure and destabilizes cytochrome c

(2017) Hongrui Meng et al.   Nature Communications

PINK1 Primes Parkin-Mediated Ubiquitination of PARIS in Dopaminergic Neuronal Survival

(2017) Yunjong Lee et al.   Cell Reports

Mitochondrial DNA Depletion in Respiratory Chain-Deficient Parkinson Disease Neurons

(2016) Anne Grünewald et al.   ANNALS OF NEUROLOGY

Functional Impairment in Miro Degradation and Mitophagy Is a Shared Feature in Familial and Sporadic Parkinson’s Disease

(2016) Chung-Han Hsieh et al.   Cell Stem Cell

A conserved retromer sorting motif is essential for mitochondrial DLP1 recycling by VPS35 in Parkinson’s disease model

(2016) Wenzhang Wang et al.   HUMAN MOLECULAR GENETICS

Reduced mitochondrial DNA copy number is a biomarker of Parkinson's disease

(2016) Angela Pyle et al.   NEUROBIOLOGY OF AGING

α-Synuclein–induced lysosomal dysfunction occurs through disruptions in protein trafficking in human midbrain synucleinopathy models

(2016) Joseph R. Mazzulli et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Defective mitochondrial DNA homeostasis in the substantia nigra in Parkinson disease

(2016) Christian Dölle et al.   Nature Communications

Reduced cerebrospinal fluid mitochondrial DNA is a biomarker for early-stage Parkinson's disease

(2015) Angela Pyle et al.   ANNALS OF NEUROLOGY

spargel, the PGC-1α homologue, in models of Parkinson disease in Drosophila melanogaster

(2015) Eric M. Merzetti et al.   BMC NEUROSCIENCE

New insights into PGC-1 coactivators: redefining their role in the regulation of mitochondrial function and beyond

(2015) Josep A. Villena   FEBS Journal

Structural and Functional Impact of Parkinson Disease-Associated Mutations in the E3 Ubiquitin Ligase Parkin

(2015) Fabienne C. Fiesel et al.   HUMAN MUTATION

Convergence of Parkin, PINK1, and α-Synuclein on Stress-induced Mitochondrial Morphological Remodeling

(2015) Kristi L. Norris et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

CHCHD2 mutations in autosomal dominant late-onset Parkinson's disease: a genome-wide linkage and sequencing study

(2015) Manabu Funayama et al.   LANCET NEUROLOGY

MNRR1 (formerly CHCHD2) is a bi-organellar regulator of mitochondrial metabolism

(2015) Siddhesh Aras et al.   MITOCHONDRION

The PINK1-PARKIN Mitochondrial Ubiquitylation Pathway Drives a Program of OPTN/NDP52 Recruitment and TBK1 Activation to Promote Mitophagy

(2015) Jin-Mi Heo et al.   MOLECULAR CELL

Overexpression of PGC-1α Influences Mitochondrial Signal Transduction of Dopaminergic Neurons

(2015) Qinyong Ye et al.   MOLECULAR NEUROBIOLOGY

Mechanism of phospho-ubiquitin-induced PARKIN activation

(2015) Tobias Wauer et al.   NATURE

The ubiquitin kinase PINK1 recruits autophagy receptors to induce mitophagy

(2015) Michael Lazarou et al.   NATURE

Parkinson's disease–associated mutant VPS35 causes mitochondrial dysfunction by recycling DLP1 complexes

(2015) Wenzhang Wang et al.   NATURE MEDICINE

Evidence for polymerase gamma, POLG1 variation in reduced mitochondrial DNA copy number in Parkinson's disease

(2015) Ya-Xing Gui et al.   PARKINSONISM & RELATED DISORDERS

Parkin loss leads to PARIS-dependent declines in mitochondrial mass and respiration

(2015) Daniel A. Stevens et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Alzheimer's and Parkinson's diseases: The prion concept in relation to assembled A , tau, and  -synuclein

(2015) M. Goedert   SCIENCE

Impaired intracellular trafficking defines early Parkinson's disease

(2015) Benjamin H.M. Hunn et al.   TRENDS IN NEUROSCIENCES

PGC-1α activity in nigral dopamine neurons determines vulnerability to α-synuclein

(2015) Carine Ciron et al.   Acta Neuropathologica Communications

PGC−1α Promoter Methylation in Parkinson’s Disease

(2015) Xiaomin Su et al.   PLoS One

Mutual exacerbation of peroxisome proliferator-activated receptor γ coactivator 1α deregulation and α-synuclein oligomerization

(2014) Judith Eschbach et al.   ANNALS OF NEUROLOGY

Parkin is activated by PINK1-dependent phosphorylation of ubiquitin at Ser65

(2014) Agne Kazlauskaite et al.   BIOCHEMICAL JOURNAL

Number and Brightness analysis of alpha-synuclein oligomerization and the associated mitochondrial morphology alterations in live cells

(2014) N. Plotegher et al.   BIOCHIMICA ET BIOPHYSICA ACTA-GENERAL SUBJECTS

Effect of resveratrol on mitochondrial function: Implications in parkin-associated familiar Parkinson's disease

(2014) Anna Ferretta et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE

Functional interplay between Parkin and Drp1 in mitochondrial fission and clearance

(2014) Lori Buhlman et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR CELL RESEARCH

α-Synuclein oligomers distinctively permeabilize complex model membranes

(2014) Anja N. D. Stefanovic et al.   FEBS Journal

Mitochondrial DNA copy number in peripheral blood cells declines with age and is associated with general health among elderly

(2014) Jonas Mengel-From et al.   HUMAN GENETICS

Parkinson's disease-linked mutations in VPS35 induce dopaminergic neurodegeneration

(2014) E. Tsika et al.   HUMAN MOLECULAR GENETICS

PINK1 phosphorylates ubiquitin to activate Parkin E3 ubiquitin ligase activity

(2014) Lesley A. Kane et al.   JOURNAL OF CELL BIOLOGY

Ubiquitin is phosphorylated by PINK1 to activate parkin

(2014) Fumika Koyano et al.   NATURE

Region specific mitochondrial impairment in mice with widespread overexpression of alpha-synuclein

(2014) Sudhakar Raja Subramaniam et al.   NEUROBIOLOGY OF DISEASE

PINK1 is degraded through the N-end rule pathway

(2013) Koji Yamano et al.   Autophagy

Isogenic Human iPSC Parkinson’s Model Shows Nitrosative Stress-Induced Dysfunction in MEF2-PGC1α Transcription

(2013) Scott D. Ryan et al.   CELL

Structure of the human Parkin ligase domain in an autoinhibited state

(2013) Tobias Wauer et al.   EMBO JOURNAL

Parkinson disease: from pathology to molecular disease mechanisms

(2013) David T. Dexter et al.   FREE RADICAL BIOLOGY AND MEDICINE

Hepatic-specific activation of peroxisome proliferator-activated receptor γ coactivator-1β protects against steatohepatitis

(2013) Elena Bellafante et al.   HEPATOLOGY

Exposure to pesticides or solvents and risk of Parkinson disease

(2013) G. Pezzoli et al.   NEUROLOGY

Identification and Rescue of  -Synuclein Toxicity in Parkinson Patient-Derived Neurons

(2013) C. Y. Chung et al.   SCIENCE

p53 Orchestrates the PGC-1α-Mediated Antioxidant Response Upon Mild Redox and Metabolic Imbalance

(2012) Katia Aquilano et al.   ANTIOXIDANTS & REDOX SIGNALING

The Role of PGC-1α in the Pathogenesis of Neurodegenerative Disorders

(2012) Krisztina Rona-Voros et al.   CURRENT DRUG TARGETS

Genetics of Parkinson disease and other movement disorders

(2012) Kishore R. Kumar et al.   CURRENT OPINION IN NEUROLOGY

Selective binding of nuclear alpha-synuclein to the PGC1alpha promoter under conditions of oxidative stress may contribute to losses in mitochondrial function: Implications for Parkinson’s disease

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

Mitofusin 2 is necessary for striatal axonal projections of midbrain dopamine neurons

(2012) Seungmin Lee et al.   HUMAN MOLECULAR GENETICS

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

LRRK2 regulates mitochondrial dynamics and function through direct interaction with DLP1

(2012) X. Wang et al.   HUMAN MOLECULAR GENETICS

Classic and New Animal Models of Parkinson's Disease

(2012) Javier Blesa et al.   JOURNAL OF BIOMEDICINE AND BIOTECHNOLOGY

Pgc-1α Overexpression Downregulates Pitx3 and Increases Susceptibility to MPTP Toxicity Associated with Decreased Bdnf

(2012) Joanne Clark et al.   PLoS One

Genetics of Parkinson's Disease

(2012) Kishore Kumar et al.   SEMINARS IN NEUROLOGY

A Mutation in VPS35, Encoding a Subunit of the Retromer Complex, Causes Late-Onset Parkinson Disease

(2011) Alexander Zimprich et al.   AMERICAN JOURNAL OF HUMAN GENETICS

VPS35 Mutations in Parkinson Disease

(2011) Carles Vilariño-Güell et al.   AMERICAN JOURNAL OF HUMAN GENETICS

Mitochondrial defect and PGC-1α dysfunction in parkin-associated familial Parkinson's disease

(2011) Consiglia Pacelli et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE

Association of PGC-1alphapolymorphisms with age of onset and risk of Parkinson's disease

(2011) Joanne Clark et al.   BMC Medical Genetics

PARIS (ZNF746) Repression of PGC-1α Contributes to Neurodegeneration in Parkinson's Disease

(2011) Joo-Ho Shin et al.   CELL

SnapShot: Mitochondrial Quality Control

(2011) Douglas R. Green et al.   CELL

Transgenic expression and activation of PGC-1α protect dopaminergic neurons in the MPTP mouse model of Parkinson’s disease

(2011) Giuseppa Mudò et al.   CELLULAR AND MOLECULAR LIFE SCIENCES

Parkin Ubiquitinates Drp1 for Proteasome-dependent Degradation

(2011) Hongxia Wang et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Imputation of sequence variants for identification of genetic risks for Parkinson's disease: a meta-analysis of genome-wide association studies

(2011)   LANCET

DJ-1 knock-down impairs astrocyte mitochondrial function

(2011) N.J. Larsen et al.   NEUROSCIENCE

A Two-Stage Meta-Analysis Identifies Several New Loci for Parkinson's Disease

(2011) et al.   PLoS Genetics

Neurodegeneration in an animal model of Parkinson's disease is exacerbated by a high-fat diet

(2010) Jill K. Morris et al.   AMERICAN JOURNAL OF PHYSIOLOGY-REGULATORY INTEGRATIVE AND COMPARATIVE PHYSIOLOGY

Nuclear Control of Respiratory Chain Expression by Nuclear Respiratory Factors and PGC-1-Related Coactivator

(2010) Richard C. Scarpulla   Annals of the New York Academy of Sciences

Loss of the Parkinson's disease-linked gene DJ-1 perturbs mitochondrial dynamics

(2010) I. Irrcher et al.   HUMAN MOLECULAR GENETICS

Tight control of mitochondrial membrane potential by cytochrome c oxidase

(2010) Consiglia Pacelli et al.   MITOCHONDRION

Reduced expression of peroxisome-proliferator activated receptor gamma coactivator-1α enhances α-synuclein oligomerization and down regulates AKT/GSK3β signaling pathway in human neuronal cells that inducibly express α-synuclein

(2010) Abdul Shukkur Ebrahim et al.   NEUROSCIENCE LETTERS

Reduced Basal Autophagy and Impaired Mitochondrial Dynamics Due to Loss of Parkinson's Disease-Associated Protein DJ-1

(2010) Guido Krebiehl et al.   PLoS One

DJ-1 is critical for mitochondrial function and rescues PINK1 loss of function

(2010) L.-Y. Hao et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

PGC-1 , A Potential Therapeutic Target for Early Intervention in Parkinson's Disease

(2010) B. Zheng et al.   Science Translational Medicine

Identification and characterization of an alternative promoter of the human PGC-1α gene

(2009) Toyo Yoshioka et al.   BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS

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

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

GCN5-mediated Transcriptional Control of the Metabolic Coactivator PGC-1β through Lysine Acetylation

(2009) Timothy J. Kelly et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Alternative mRNA Splicing Produces a Novel Biologically Active Short Isoform of PGC-1α

(2009) Yubin Zhang et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

p38 mitogen-activated protein kinase and calcium channels mediate signaling in depolarization-induced activation of peroxisome proliferator-activated receptor gamma coactivator-1α in neurons

(2009) Huan Ling Liang et al.   JOURNAL OF NEUROSCIENCE RESEARCH

The biology of PGC-1α and its therapeutic potential

(2009) Christoph Handschin   TRENDS IN PHARMACOLOGICAL SCIENCES

Genome-wide Coactivation Analysis of PGC-1α Identifies BAF60a as a Regulator of Hepatic Lipid Metabolism

(2008) Siming Li et al.   Cell Metabolism

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

Mitochondrial localization of DJ-1 leads to enhanced neuroprotection

(2008) Eunsung Junn et al.   JOURNAL OF NEUROSCIENCE RESEARCH

Mitochondrial function in Parkinson’s disease cybrids containing an nt2 neuron-like nuclear background

(2008) A. Raquel F. Esteves et al.   MITOCHONDRION

AMP-Activated Protein Kinase-Regulated Activation of the PGC-1α Promoter in Skeletal Muscle Cells

(2008) Isabella Irrcher et al.   PLoS One

Control of OXPHOS efficiency by complex I in brain mitochondria

(2007) Tiziana Cocco et al.   NEUROBIOLOGY OF AGING