ULK overexpression mitigates motor deficits and neuropathology in mouse models of Machado-Joseph disease

ULK1 and ULK2 are less redundant than previously thought: computational analysis uncovers distinct regulation and functions of these autophagy induction proteins

(2020) Amanda Demeter et al.   Scientific Reports

Ulk1 Governs Nerve Growth Factor/TrkA Signaling by Mediating Rab5 GTPase Activation in Porcine Hemagglutinating Encephalomyelitis Virus-Induced Neurodegenerative Disorders

(2018) Zi Li et al.   JOURNAL OF VIROLOGY

Transcriptional profiling and biomarker identification reveal tissue specific effects of expanded ataxin-3 in a spinocerebellar ataxia type 3 mouse model

(2018) Lodewijk J. A. Toonen et al.   Molecular Neurodegeneration

Repeated Mesenchymal Stromal Cells Treatment Sustainably Alleviates Machado-Joseph Disease

(2018) Catarina Oliveira Miranda et al.   MOLECULAR THERAPY

Unravelling Endogenous MicroRNA System Dysfunction as a New Pathophysiological Mechanism in Machado-Joseph Disease

(2017) Vitor Carmona et al.   MOLECULAR THERAPY

Polyglutamine tracts regulate beclin 1-dependent autophagy

(2017) Avraham Ashkenazi et al.   NATURE

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

(2017) Annie Sittler et al.   NEUROPATHOLOGY

Guidelines for the use and interpretation of assays for monitoring autophagy (3rd edition)

(2016) Daniel J Klionsky et al.   Autophagy

Intravenous administration of brain-targeted stable nucleic acid lipid particles alleviates Machado-Joseph disease neurological phenotype

(2016) Mariana Conceição et al.   BIOMATERIALS

ULK1-mediated phosphorylation of ATG14 promotes autophagy and is impaired in Huntington’s disease models

(2016) Mitchell S. Wold et al.   Molecular Neurodegeneration

Expansion of the Gene Ontology knowledgebase and resources

(2016)   NUCLEIC ACIDS RESEARCH

Fibroblasts of Machado Joseph Disease patients reveal autophagy impairment

(2016) Isabel Onofre et al.   Scientific Reports

Re-establishing ataxin-2 downregulates translation of mutant ataxin-3 and alleviates Machado–Joseph disease

(2015) Clévio Nóbrega et al.   BRAIN

Neuropeptide Y mitigates neuropathology and motor deficits in mouse models of Machado–Joseph disease

(2015) Joana Duarte-Neves et al.   HUMAN MOLECULAR GENETICS

Huntingtin functions as a scaffold for selective macroautophagy

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

Proteotoxic Stress Induces Phosphorylation of p62/SQSTM1 by ULK1 to Regulate Selective Autophagic Clearance of Protein Aggregates

(2015) Junghyun Lim et al.   PLoS Genetics

Transplantation of cerebellar neural stem cells improves motor coordination and neuropathology in Machado-Joseph disease mice

(2014) Liliana S. Mendonça et al.   BRAIN

The Regulation of Autophagosome Dynamics by Huntingtin and HAP1 Is Disrupted by Expression of Mutant Huntingtin, Leading to Defective Cargo Degradation

(2014) Y. C. Wong et al.   JOURNAL OF NEUROSCIENCE

RNA Interference Mitigates Motor and Neuropathological Deficits in a Cerebellar Mouse Model of Machado-Joseph Disease

(2014) Clévio Nóbrega et al.   PLoS One

Caffeine and adenosine A2Areceptor inactivation decrease striatal neuropathology in a lentiviral-based model of Machado-Joseph disease

(2013) Nélio Gonçalves et al.   ANNALS OF NEUROLOGY

Distinct functions ofUlk1andUlk2in the regulation of lipid metabolism in adipocytes

(2013) Seung-Hyun Ro et al.   Autophagy

Beclin 1 mitigates motor and neuropathological deficits in genetic mouse models of Machado–Joseph disease

(2013) Isabel Nascimento-Ferreira et al.   BRAIN

The machinery of macroautophagy

(2013) Yuchen Feng et al.   CELL RESEARCH

Inhibition of Autophagy via p53-Mediated Disruption of ULK1 in a SCA7 Polyglutamine Disease Model

(2013) Xin Yu et al.   JOURNAL OF MOLECULAR NEUROSCIENCE

ULK1 induces autophagy by phosphorylating Beclin-1 and activating VPS34 lipid kinase

(2013) Ryan C. Russell et al.   NATURE CELL BIOLOGY

Primer-BLAST: A tool to design target-specific primers for polymerase chain reaction

(2012) Jian Ye et al.   BMC BIOINFORMATICS

Calpastatin-mediated inhibition of calpains in the mouse brain prevents mutant ataxin 3 proteolysis, nuclear localization and aggregation, relieving Machado-Joseph disease

(2012) A. T. Simoes et al.   BRAIN

Binding of the Atg1/ULK1 kinase to the ubiquitin-like protein Atg8 regulates autophagy

(2012) Claudine Kraft et al.   EMBO JOURNAL

Autophagy-related proteins (p62, NBR1 and LC3) in intranuclear inclusions in neurodegenerative diseases

(2012) Fumiaki Mori et al.   NEUROSCIENCE LETTERS

The Role of Atg Proteins in Autophagosome Formation

(2011) Noboru Mizushima et al.   Annual Review of Cell and Developmental Biology

The requirement of uncoordinated 51-like kinase 1 (ULK1) and ULK2 in the regulation of autophagy

(2011) Eun-Ju Lee et al.   Autophagy

Overexpression of the autophagic beclin-1 protein clears mutant ataxin-3 and alleviates Machado–Joseph disease

(2011) Isabel Nascimento-Ferreira et al.   BRAIN

Ebola virus entry requires the cholesterol transporter Niemann–Pick C1

(2011) Jan E. Carette et al.   NATURE

Ammonia-induced autophagy is independent of ULK1/ULK2 kinases

(2011) H. Cheong et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

ULK1·ATG13·FIP200 Complex Mediates mTOR Signaling and Is Essential for Autophagy

(2009) Ian G. Ganley et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Ulk1 plays a critical role in the autophagic clearance of mitochondria and ribosomes during reticulocyte maturation

(2008) M. Kundu et al.   BLOOD

Characterization of mutant mice that express polyglutamine in cerebellar Purkinje cells

(2008) Miho Oue et al.   BRAIN RESEARCH

Lentivector-mediated rescue from cerebellar ataxia in a mouse model of spinocerebellar ataxia

(2008) Takashi Torashima et al.   EMBO REPORTS

Striatal and nigral pathology in a lentiviral rat model of Machado-Joseph disease

(2008) Sandro Alves et al.   HUMAN MOLECULAR GENETICS

Analysis of segregation patterns in Machado–Joseph disease pedigrees

(2008) Conceição Bettencourt et al.   JOURNAL OF HUMAN GENETICS

Kinase-Inactivated ULK Proteins Inhibit Autophagy via Their Conserved C-Terminal Domains Using an Atg13-Independent Mechanism

(2008) E. Y. W. Chan et al.   MOLECULAR AND CELLULAR BIOLOGY

Polyglutamine-expanded ataxin-3 causes cerebellar dysfunction of SCA3 transgenic mice by inducing transcriptional dysregulation

(2008) An-Hsun Chou et al.   NEUROBIOLOGY OF DISEASE