Sumoylation of Critical Proteins in Amyotrophic Lateral Sclerosis: Emerging Pathways of Pathogenesis

Rodent models of amyotrophic lateral sclerosis

(2013) Philip McGoldrick et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE

Disease-Specific iPS Cell Models in Neuroscience

(2013) M. Peitz et al.   CURRENT MOLECULAR MEDICINE

Motor neurons and glia exhibit specific individualized responses to TDP-43 expression in a Drosophila model of amyotrophic lateral sclerosis

(2013) P. S. Estes et al.   Disease Models & Mechanisms

Overexpression of human mutated G93A SOD1 changes dynamics of the ER mitochondria calcium cycle specifically in mouse embryonic motor neurons

(2013) Janin Lautenschläger et al.   EXPERIMENTAL NEUROLOGY

Hdac6 deletion delays disease progression in the SOD1G93A mouse model of ALS

(2013) Ines Taes et al.   HUMAN MOLECULAR GENETICS

Riluzole increases glutamate uptake by cultured C6 astroglial cells

(2013) Oscar P. Dall’Igna et al.   INTERNATIONAL JOURNAL OF DEVELOPMENTAL NEUROSCIENCE

HDAC6 Regulates Mutant SOD1 Aggregation through Two SMIR Motifs and Tubulin Acetylation

(2013) Jozsef Gal et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Mutations in prion-like domains in hnRNPA2B1 and hnRNPA1 cause multisystem proteinopathy and ALS

(2013) Hong Joo Kim et al.   NATURE

Review: Microglia in protein aggregation disorders: friend or foe?

(2013) S. M. Gentleman   NEUROPATHOLOGY AND APPLIED NEUROBIOLOGY

Sodium Valproate Alleviates Neurodegeneration in SCA3/MJD via Suppressing Apoptosis and Rescuing the Hypoacetylation Levels of Histone H3 and H4

(2013) Jiping Yi et al.   PLoS One

PIASγ Enhanced SUMO-2 Modification of Nurr1 Activation-Function-1 Domain Limits Nurr1 Transcriptional Synergy

(2013) Cristian Arredondo et al.   PLoS One

Neural Progenitors Derived From Human Induced Pluripotent Stem Cells Survive and Differentiate Upon Transplantation Into a Rat Model of Amyotrophic Lateral Sclerosis

(2013) Iuliana Ristea Popescu et al.   Stem Cells Translational Medicine

The impact of clinical factors, riluzole and therapeutic interventions on ALS survival: A population based study in Modena, Italy

(2013) Eleni Georgoulopoulou et al.   Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration

Mixed tau, TDP-43 and p62 pathology in FTLD associated with a C9ORF72 repeat expansion and p.Ala239Thr MAPT (tau) variant

(2012) Andrew King et al.   ACTA NEUROPATHOLOGICA

Neuroimaging signatures of frontotemporal dementia genetics: C9ORF72, tau, progranulin and sporadics

(2012) J. L. Whitwell et al.   BRAIN

Sumoylation of SAE2 C Terminus Regulates SAE Nuclear Localization

(2012) Khue Truong et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

FUS/TLS-immunoreactive Neuronal and Glial Cell Inclusions Increase With Disease Duration in Familial Amyotrophic Lateral Sclerosis With an R521CFUS/TLSMutation

(2012) Naoki Suzuki et al.   JOURNAL OF NEUROPATHOLOGY AND EXPERIMENTAL NEUROLOGY

Abnormal Regenerative Responses and Impaired Axonal Outgrowth after Nerve Crush in TDP-43 Transgenic Mouse Models of Amyotrophic Lateral Sclerosis

(2012) V. Swarup et al.   JOURNAL OF NEUROSCIENCE

Inflammation and neurovascular changes in amyotrophic lateral sclerosis

(2012) M.C. Evans et al.   MOLECULAR AND CELLULAR NEUROSCIENCE

Function and regulation of SUMO proteases

(2012) Christopher M. Hickey et al.   NATURE REVIEWS MOLECULAR CELL BIOLOGY

E6-AP association promotes SOD1 aggresomes degradation and suppresses toxicity

(2012) Amit Mishra et al.   NEUROBIOLOGY OF AGING

Frontotemporal lobar degeneration with TDP-43 proteinopathy and chromosome 9p repeat expansion inC9ORF72: clinicopathologic correlation

(2012) Eileen H. Bigio et al.   NEUROPATHOLOGY

Ultrastructure of ubiquitin-positive, TDP-43-negative neuronal inclusions in cerebral cortex of C9ORF72-linked frontotemporal lobar degeneration/amyotrophic lateral sclerosis

(2012) Wenlang Lin et al.   NEUROPATHOLOGY

Regionally different immunoreactivity for Smurf2 and pSmad2/3 in TDP-43-positive inclusions of amyotrophic lateral sclerosis

(2012) M. Nakamura et al.   NEUROPATHOLOGY AND APPLIED NEUROBIOLOGY

Reduced protein O-glycosylation in the nervous system of the mutant SOD1 transgenic mouse model of amyotrophic lateral sclerosis

(2012) Xiaoyang Shan et al.   NEUROSCIENCE LETTERS

Mutant TDP-43 and FUS Cause Age-Dependent Paralysis and Neurodegeneration in C. elegans

(2012) Alexandra Vaccaro et al.   PLoS One

Knockdown of the Drosophila Fused in Sarcoma (FUS) Homologue Causes Deficient Locomotive Behavior and Shortening of Motoneuron Terminal Branches

(2012) Hiroshi Sasayama et al.   PLoS One

An over-oxidized form of superoxide dismutase found in sporadic amyotrophic lateral sclerosis with bulbar onset shares a toxic mechanism with mutant SOD1

(2012) S. Guareschi et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

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

(2012) Vanita Chopra et al.   Cell Reports

Glutathionylation at Cys-111 Induces Dissociation of Wild Type and FALS Mutant SOD1 Dimers

(2011) Rachel L. Redler et al.   BIOCHEMISTRY

Riluzole is a potent drug to protect neonatal rat hypoglossal motoneurons in vitro from excitotoxicity due to glutamate uptake block

(2011) Alessandra Cifra et al.   EUROPEAN JOURNAL OF NEUROSCIENCE

Treatment with trichostatin A initiated after disease onset delays disease progression and increases survival in a mouse model of amyotrophic lateral sclerosis

(2011) Young-Eun Yoo et al.   EXPERIMENTAL NEUROLOGY

Motor neuron impairment mediated by a sumoylated fragment of the glial glutamate transporter EAAT2

(2011) Emily Foran et al.   GLIA

Caveolin-3 Undergoes SUMOylation by the SUMO E3 Ligase PIASy

(2011) Stephen R. Fuhs et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Mutations in UBQLN2 cause dominant X-linked juvenile and adult-onset ALS and ALS/dementia

(2011) Han-Xiang Deng et al.   NATURE

Mutational analysis of the VCP gene in Parkinson's disease

(2011) Elisa Majounie et al.   NEUROBIOLOGY OF AGING

Constructing and Deconstructing Stem Cell Models of Neurological Disease

(2011) Steve S.W. Han et al.   NEURON

Protective effects of valproic acid on the nigrostriatal dopamine system in a 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine mouse model of Parkinson's disease

(2011) S.K. Kidd et al.   NEUROSCIENCE

Expression of human FUS/TLS in yeast leads to protein aggregation and cytotoxicity, recapitulating key features of FUS proteinopathy

(2011) Kazuo Fushimi et al.   Protein & Cell

Rate of familial amyotrophic lateral sclerosis: a systematic review and meta-analysis

(2010) S. Byrne et al.   JOURNAL OF NEUROLOGY NEUROSURGERY AND PSYCHIATRY

Cytoplasmic Mislocalization of TDP-43 Is Toxic to Neurons and Enhanced by a Mutation Associated with Familial Amyotrophic Lateral Sclerosis

(2010) S. J. Barmada et al.   JOURNAL OF NEUROSCIENCE

Multiplex SILAC Analysis of a Cellular TDP-43 Proteinopathy Model Reveals Protein Inclusions Associated with SUMOylation and Diverse Polyubiquitin Chains

(2010) Nicholas T. Seyfried et al.   MOLECULAR & CELLULAR PROTEOMICS

Fusgene mutations in familial and sporadic amyotrophic lateral sclerosis

(2010) Rosa Rademakers et al.   MUSCLE & NERVE

Mutations of optineurin in amyotrophic lateral sclerosis

(2010) Hirofumi Maruyama et al.   NATURE

Exome Sequencing Reveals VCP Mutations as a Cause of Familial ALS

(2010) Janel O. Johnson et al.   NEURON

Ubiquitinated, p62 immunopositive cerebellar cortical neuronal inclusions are evident across the spectrum of TDP-43 proteinopathies but are only rarely additionally immunopositive for phosphorylation-dependent TDP-43

(2010) Andrew King et al.   NEUROPATHOLOGY

Amyotrophic lateral sclerosis and frontotemporal lobar degeneration: A spectrum of TDP-43 proteinopathies

(2010) Felix Geser et al.   NEUROPATHOLOGY

Familial amyotrophic lateral sclerosis is associated with a mutation in D-amino acid oxidase

(2010) J. Mitchell et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

In vitroneurotoxic properties and excitatory aminoacids concentration in the cerebrospinal fluid of amyotrophic lateral sclerosis patients. Relationship with the degree of certainty of disease diagnoses

(2009) M. L. Fiszman et al.   ACTA NEUROLOGICA SCANDINAVICA

Glutamate Transporters and the Excitotoxic Path to Motor Neuron Degeneration in Amyotrophic Lateral Sclerosis

(2009) Emily Foran et al.   ANTIOXIDANTS & REDOX SIGNALING

Impairment of mitochondrial calcium handling in a mtSOD1 cell culture model of motoneuron disease

(2009) Manoj Jaiswal et al.   BMC NEUROSCIENCE

SUMOylation of the mitochondrial fission protein Drp1 occurs at multiple nonconsensus sites within the B domain and is linked to its activity cycle

(2009) Claudia Figueroa-Romero et al.   FASEB JOURNAL

Small Ubiquitin-like Modifier (SUMO) Modification of the Androgen Receptor Attenuates Polyglutamine-mediated Aggregation

(2009) Sarmistha Mukherjee et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

A role for motoneuron subtype–selective ER stress in disease manifestations of FALS mice

(2009) Smita Saxena et al.   NATURE NEUROSCIENCE

Presynaptic Regulation of Astroglial Excitatory Neurotransmitter Transporter GLT1

(2009) Yongjie Yang et al.   NEURON

Phenylbutyrate Ameliorates Cognitive Deficit and Reduces Tau Pathology in an Alzheimer's Disease Mouse Model

(2009) Ana Ricobaraza et al.   NEUROPSYCHOPHARMACOLOGY

Ebp1 sumoylation, regulated by TLS/FUS E3 ligase, is required for its anti-proliferative activity

(2009) S-M Oh et al.   ONCOGENE

Mutations in the FUS/TLS Gene on Chromosome 16 Cause Familial Amyotrophic Lateral Sclerosis

(2009) T. J. Kwiatkowski et al.   SCIENCE

Protein interactions in the sumoylation cascade - lessons from X-ray structures

(2008) Zhongshu Tang et al.   FEBS Journal

Detergent-insoluble Aggregates Associated with Amyotrophic Lateral Sclerosis in Transgenic Mice Contain Primarily Full-length, Unmodified Superoxide Dismutase-1

(2008) Bryan F. Shaw et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

SUMO-Specific Protease 1 (SENP1) Reverses the Hormone-Augmented SUMOylation of Androgen Receptor and Modulates Gene Responses in Prostate Cancer Cells

(2008) Sanna Kaikkonen et al.   MOLECULAR ENDOCRINOLOGY