Mitochondrial Morphology, Function and Homeostasis Are Impaired by Expression of an N-terminal Calpain Cleavage Fragment of Ataxin-3

Accumulation of Mitochondrial DNA Common Deletion Since The Preataxic Stage of Machado-Joseph Disease

(2018) Mafalda Raposo et al.   MOLECULAR NEUROBIOLOGY

Mass spectrometry analyses of normal and polyglutamine expanded ataxin-3 reveal novel interaction partners involved in mitochondrial function

(2018) Line V. Kristensen et al.   NEUROCHEMISTRY INTERNATIONAL

Generation of an induced pluripotent stem cell line from a patient with spinocerebellar ataxia type 3 (SCA3): HIHCNi002-A

(2018) Stefanie Nicole Hayer et al.   Stem Cell Research

Deregulation of autophagy in postmortem brains of Machado-Joseph disease patients

(2017) Annie Sittler et al.   NEUROPATHOLOGY

The Truncated C-terminal Fragment of Mutant ATXN3 Disrupts Mitochondria Dynamics in Spinocerebellar Ataxia Type 3 Models

(2017) Jung-Yu Hsu et al.   Frontiers in Molecular Neuroscience

Loss of Mitochondrial Function Impairs Lysosomes

(2016) Julie Demers-Lamarche et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Differential mtDNA Damage Patterns in a Transgenic Mouse Model of Machado–Joseph Disease (MJD/SCA3)

(2014) Amanda Ramos et al.   JOURNAL OF MOLECULAR NEUROSCIENCE

From Pathways to Targets: Understanding the Mechanisms behind Polyglutamine Disease

(2014) Jonasz Jeremiasz Weber et al.   Biomed Research International

The lysosome: from waste bag to potential therapeutic target

(2013) Hanna Appelqvist et al.   Journal of Molecular Cell Biology

Crosstalk between the UPR and autophagy pathway contributes to handling cellular stress in neurodegenerative disease

(2012) René L. Vidal et al.   Autophagy

Shaping the role of mitochondria in the pathogenesis of Huntington's disease

(2012) Veronica Costa et al.   EMBO JOURNAL

Targeting the UPR transcription factor XBP1 protects against Huntington's disease through the regulation of FoxO1 and autophagy

(2012) Rene L. Vidal et al.   HUMAN MOLECULAR GENETICS

Sustained expression of PGC-1  in the rat nigrostriatal system selectively impairs dopaminergic function

(2012) C. Ciron et al.   HUMAN MOLECULAR GENETICS

PGC1  and mitochondrial metabolism - emerging concepts and relevance in ageing and neurodegenerative disorders

(2012) S. Austin et al.   JOURNAL OF CELL SCIENCE

Automated Behavioral Phenotyping Reveals Presymptomatic Alterations in a SCA3 Genetrap Mouse Model

(2012) Jeannette Hübener et al.   Journal of Genetics and Genomics

Primary Skin Fibroblasts as a Model of Parkinson's Disease

(2012) Georg Auburger et al.   MOLECULAR NEUROBIOLOGY

Directed differentiation of human pluripotent stem cells to cerebral cortex neurons and neural networks

(2012) Yichen Shi et al.   Nature Protocols

Patterns of Mitochondrial DNA Damage in Blood and Brain Tissues of a Transgenic Mouse Model of Machado-Joseph Disease

(2012) Nadiya Kazachkova et al.   Neurodegenerative Diseases

Compromised mitochondrial complex II in models of Machado–Joseph disease

(2011) Mário N. Laço et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE

N-terminal ataxin-3 causes neurological symptoms with inclusions, endoplasmic reticulum stress and ribosomal dislocation

(2011) Jeannette Hübener et al.   BRAIN

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

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

(2011) Wenjun Song et al.   NATURE MEDICINE

Truncated Peroxisome Proliferator-Activated Receptor-γ Coactivator 1α Splice Variant Is Severely Altered in Huntington’s Disease

(2011) Ashu Johri et al.   Neurodegenerative Diseases

The Interplay Between Mitochondrial Dynamics and Mitophagy

(2010) Gilad Twig et al.   ANTIOXIDANTS & REDOX SIGNALING

Mitochondrial fission and cristae disruption increase the response of cell models of Huntington's disease to apoptotic stimuli

(2010) Veronica Costa et al.   EMBO Molecular Medicine

Mitochondrial dysfunction in neurodegenerative diseases and cancer

(2010) Michelle Barbi de Moura et al.   ENVIRONMENTAL AND MOLECULAR MUTAGENESIS

The Machado–Joseph disease-associated mutant form of ataxin-3 regulates parkin ubiquitination and stability

(2010) Thomas M. Durcan et al.   HUMAN MOLECULAR GENETICS

A mitochondrial ubiquitin ligase MITOL controls cell toxicity of polyglutamine-expanded protein

(2010) Ayumu Sugiura et al.   MITOCHONDRION

p53 activation mediates polyglutamine-expanded ataxin-3 upregulation of Bax expression in cerebellar and pontine nuclei neurons

(2010) An-Hsun Chou et al.   NEUROCHEMISTRY INTERNATIONAL

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

Deletion of the Huntingtin Polyglutamine Stretch Enhances Neuronal Autophagy and Longevity in Mice

(2010) Shuqiu Zheng et al.   PLoS Genetics

PGC-1α, SIRT1 and AMPK, an energy sensing network that controls energy expenditure

(2009) Carles Cantó et al.   CURRENT OPINION IN LIPIDOLOGY

Loss of PINK1 Function Promotes Mitophagy through Effects on Oxidative Stress and Mitochondrial Fission

(2009) Ruben K. Dagda et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Decreased antioxidant enzyme activity and increased mitochondrial DNA damage in cellular models of Machado-Joseph disease

(2009) Ya-Chun Yu et al.   JOURNAL OF NEUROSCIENCE RESEARCH

SCA3: Neurological features, pathogenesis and animal models

(2008) Olaf Riess et al.   CEREBELLUM

Parkin is recruited selectively to impaired mitochondria and promotes their autophagy

(2008) Derek Narendra et al.   JOURNAL OF CELL BIOLOGY

Rapamycin Inhibits Polyglutamine Aggregation Independently of Autophagy by Reducing Protein Synthesis

(2008) M. A. King et al.   MOLECULAR PHARMACOLOGY

Study of subcellular localization and proteolysis of ataxin-3

(2008) Chiara Pozzi et al.   NEUROBIOLOGY OF DISEASE