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Robert H. Baloh
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TDP-1/TDP-43 Regulates Stress Signaling and Age-Dependent Proteotoxicity in Caenorhabditis elegans
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TDP-43 neurotoxicity and protein aggregation modulated by heat shock factor and insulin/IGF-1 signaling
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HUMAN MOLECULAR GENETICS (2011)
A Drosophila model of FUS-related neurodegeneration reveals genetic interaction between FUS and TDP-43
Nicholas A. Lanson et al.
HUMAN MOLECULAR GENETICS (2011)
Wild-type and A315T mutant TDP-43 exert differential neurotoxicity in a Drosophila model of ALS
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HUMAN MOLECULAR GENETICS (2011)
TAR DNA-binding protein 43 (TDP-43) regulates stress granule dynamics via differential regulation of G3BP and TIA-1
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HUMAN MOLECULAR GENETICS (2011)
Identification of Neuronal RNA Targets of TDP-43-containing Ribonucleoprotein Complexes
Chantelle F. Sephton et al.
JOURNAL OF BIOLOGICAL CHEMISTRY (2011)
The ALS-associated proteins FUS and TDP-43 function together to affect Drosophila locomotion and life span
Ji-Wu Wang et al.
JOURNAL OF CLINICAL INVESTIGATION (2011)
Structure, Function, and Mechanism of Progranulin; the Brain and Beyond
Huishi Toh et al.
JOURNAL OF MOLECULAR NEUROSCIENCE (2011)
Expanded GGGGCC Hexanucleotide Repeat in Noncoding Region of C9ORF72 Causes Chromosome 9p-Linked FTD and ALS
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NEURON (2011)
A neurodegenerative disease mutation that accelerates the clearance of apoptotic cells
Aimee W. Kao et al.
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2011)
FUS and TARDBP but Not SOD1 Interact in Genetic Models of Amyotrophic Lateral Sclerosis
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PLOS GENETICS (2011)
Progranulin deficiency leads to enhanced cell vulnerability and TDP-43 translocation in primary neuronal cultures
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BRAIN RESEARCH (2010)
Gain and loss of function of ALS-related mutations of TARDBP (TDP-43) cause motor deficits in vivo
Edor Kabashi et al.
HUMAN MOLECULAR GENETICS (2010)
Neurotoxic effects of TDP-43 overexpression in C-elegans
Peter E. A. Ash et al.
HUMAN MOLECULAR GENETICS (2010)
Mutant FUS proteins that cause amyotrophic lateral sclerosis incorporate into stress granules
Daryl A. Bosco et al.
HUMAN MOLECULAR GENETICS (2010)
TDP-43 and FUS/TLS: emerging roles in RNA processing and neurodegeneration
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HUMAN MOLECULAR GENETICS (2010)
Interaction with Polyglutamine Aggregates Reveals a Q/N-rich Domain in TDP-43
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JOURNAL OF BIOLOGICAL CHEMISTRY (2010)
Amyotrophic Lateral Sclerosis-associated Proteins TDP-43 and FUS/TLS Function in a Common Biochemical Complex to Co-regulate HDAC6 mRNA
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JOURNAL OF BIOLOGICAL CHEMISTRY (2010)
Protein homeostasis and aging in neurodegeneration
Peter M. Douglas et al.
JOURNAL OF CELL BIOLOGY (2010)
Parkinson's disease-associated DJ-1 modulates innate immunity signaling in Caenorhabditis elegans
Elena M. Cornejo Castro et al.
JOURNAL OF NEURAL TRANSMISSION (2010)
Cytoplasmic Mislocalization of TDP-43 Is Toxic to Neurons and Enhanced by a Mutation Associated with Familial Amyotrophic Lateral Sclerosis
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Phosphorylation Promotes Neurotoxicity in a Caenorhabditis elegans Model of TDP-43 Proteinopathy
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JOURNAL OF NEUROSCIENCE (2010)
The evidence for altered RNA metabolism in amyotrophic lateral sclerosis (ALS)
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JOURNAL OF THE NEUROLOGICAL SCIENCES (2010)
Progranulin modulates zebrafish motoneuron development in vivo and rescues truncation defects associated with knockdown of Survival motor neuron 1
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MOLECULAR NEURODEGENERATION (2010)
Phosphorylated and cleaved TDP-43 in ALS, FTLD and other neurodegenerative disorders and in cellular models of TDP-43 proteinopathy
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NEUROPATHOLOGY (2010)
The RNA-binding protein FUS/TLS is a common aggregate-interacting protein in polyglutamine diseases
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NEUROSCIENCE RESEARCH (2010)
Progranulin is Neurotrophic In Vivo and Protects against a Mutant TDP-43 Induced Axonopathy
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A Drosophila model for TDP-43 proteinopathy
Yan Li et al.
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2010)
TDP-43 transgenic mice develop spastic paralysis and neuronal inclusions characteristic of ALS and frontotemporal lobar degeneration
Hans Wils et al.
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2010)
Phosphorylated TDP-43 in Alzheimer's disease and dementia with Lewy bodies
Tetsuaki Arai et al.
ACTA NEUROPATHOLOGICA (2009)
Progranulin is expressed within motor neurons and promotes neuronal cell survival
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BMC NEUROSCIENCE (2009)
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Paul Anderson et al.
CURRENT BIOLOGY (2009)
Proteolytic processing of TAR DNA binding protein-43 by caspases produces C-terminal fragments with disease defining properties independent of progranulin
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JOURNAL OF NEUROCHEMISTRY (2009)
TDP-43 is recruited to stress granules in conditions of oxidative insult
Claudia Colombrita et al.
JOURNAL OF NEUROCHEMISTRY (2009)
Aberrant cleavage of TDP-43 enhances aggregation and cellular toxicity
Yong-Jie Zhang et al.
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2009)
Colocalization of Transactivation-Responsive DNA-Binding Protein 43 and Huntingtin in Inclusions of Huntington Disease
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JOURNAL OF NEUROPATHOLOGY AND EXPERIMENTAL NEUROLOGY (2008)
TDP-43 regulates retinoblastoma protein phosphorylation through the repression of cyclin-dependent kinase 6 expression
Youhna M. Ayala et al.
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2008)
Missense mutations in the progranulin gene linked to frontotemporal lobar degeneration with ubiquitin-immunoreactive inclusions reduce progranulin production and secretion
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JOURNAL OF BIOLOGICAL CHEMISTRY (2008)
HDAC6 at the intersection of autophagy, the ubiquitin-proteasome system and neurodegeneration
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Huntingtin-interacting protein 1 influences worm and mouse presynaptic function and protects Caenorhabditis elegans neurons against mutant polyglutamine toxicity
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Progranulin mediates caspase-dependent cleavage of TAR DNA binding protein-43
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TDP43 is a human low molecular weight neurofilament (hNFL) mRNA-binding protein
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MOLECULAR AND CELLULAR NEUROSCIENCE (2007)
Mutations in progranulin are a major cause of ubiquitin-positive frontotemporal lobar degeneration
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HUMAN MOLECULAR GENETICS (2006)
Ubiquitinated TDP-43 in frontotemporal lobar degeneration and amyotrophic lateral sclerosis
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SCIENCE (2006)
Mutations in progranulin cause tau-negative frontotemporal dementia linked to chromosome 17
Matt Baker et al.
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HDAC6 and microtubules are required for autophagic degradation of aggregated Huntingtin
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JOURNAL OF BIOLOGICAL CHEMISTRY (2005)
Resveratrol rescues mutant polyglutamine cytotoxicity in nematode and mammalian neurons
JA Parker et al.
NATURE GENETICS (2005)
Nuclear factor TDP-43 binds to the polymorphic TG repeats in CFTR intron 8 and causes skipping of Exon 9: A functional link with disease penetrance
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AMERICAN JOURNAL OF HUMAN GENETICS (2004)
Polyglutamine protein aggregation and toxicity are linked to the cellular stress response
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HUMAN MOLECULAR GENETICS (2003)
Expanded polyglutamines in Caenorhabditis elegans cause axonal abnormalities and severe dysfunction of PLM mechanosensory neurons without cell death
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PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2001)
Dominant phenotypes produced by the HD mutation in STHdhQ111 striatal cells
F Trettel et al.
HUMAN MOLECULAR GENETICS (2000)
Identification of genes that modify ataxin-1-induced neurodegeneration
P Fernandez-Funez et al.
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