Neurons selectively targeted in frontotemporal dementia reveal early stage TDP-43 pathobiology

The vulnerability of spinal motoneurons and soma size plasticity in a mouse model of amyotrophic lateral sclerosis

(2018) S. Shekar Dukkipati et al.   JOURNAL OF PHYSIOLOGY-LONDON

Network architecture underlying basal autonomic outflow: Evidence from frontotemporal dementia

(2018) Virginia E. Sturm et al.   JOURNAL OF NEUROSCIENCE

von Economo Neuron Density and Thalamus Volumes in Behavioral Deficits in Frontotemporal Dementia Cases with and without a C9ORF72 Repeat Expansion

(2017) Yue Yang et al.   JOURNAL OF ALZHEIMERS DISEASE

Anterior Cingulate Cortex TDP-43 Pathology in Sporadic Amyotrophic Lateral Sclerosis

(2017) Heiko Braak et al.   JOURNAL OF NEUROPATHOLOGY AND EXPERIMENTAL NEUROLOGY

Tdp-43 cryptic exons are highly variable between cell types

(2017) Yun Ha Jeong et al.   Molecular Neurodegeneration

Diagnosis and management of dementia with Lewy bodies

(2017) Ian G. McKeith et al.   NEUROLOGY

Network degeneration and dysfunction in presymptomatic C9ORF72 expansion carriers

(2017) Suzee E. Lee et al.   NeuroImage-Clinical

Pathological TDP-43 changes in Betz cells differ from those in bulbar and spinal α-motoneurons in sporadic amyotrophic lateral sclerosis

(2016) Heiko Braak et al.   ACTA NEUROPATHOLOGICA

Nuclear trafficking in amyotrophic lateral sclerosis and frontotemporal lobar degeneration

(2016) Sonja Prpar Mihevc et al.   BRAIN

Timing and significance of pathological features inC9orf72expansion-associated frontotemporal dementia

(2016) Sarat C. Vatsavayai et al.   BRAIN

Von Economo Neurons and Fork Cells: A Neurochemical Signature Linked to Monoaminergic Function

(2016) Anke A Dijkstra et al.   CEREBRAL CORTEX

Extensive cryptic splicing upon loss of RBM17 and TDP43 in neurodegeneration models

(2016) Qiumin Tan et al.   HUMAN MOLECULAR GENETICS

Dominant hemisphere lateralization of cortical parasympathetic control as revealed by frontotemporal dementia

(2016) Christine C. Guo et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

TDP-43 aggregation mirrors TDP-43 knockdown, affecting the expression levels of a common set of proteins

(2016) S. Prpar Mihevc et al.   Scientific Reports

Functional recovery in new mouse models of ALS/FTLD after clearance of pathological cytoplasmic TDP-43

(2015) Adam K. Walker et al.   ACTA NEUROPATHOLOGICA

Proteomic analyses reveal that loss of TDP-43 affects RNA processing and intracellular transport

(2015) M. Štalekar et al.   NEUROSCIENCE

TDP-43 repression of nonconserved cryptic exons is compromised in ALS-FTD

(2015) J. P. Ling et al.   SCIENCE

Human iPSC-derived motoneurons harbouring TARDBP or C9ORF72 ALS mutations are dysfunctional despite maintaining viability

(2015) Anna-Claire Devlin et al.   Nature Communications

Cortical Function in Asymptomatic Carriers and Patients WithC9orf72Amyotrophic Lateral Sclerosis

(2015) Nimeshan Geevasinga et al.   JAMA Neurology

Network Dysfunction in Alzheimer’s Disease and Frontotemporal Dementia: Implications for Psychiatry

(2014) Juan Zhou et al.   BIOLOGICAL PSYCHIATRY

Reversible Behavioral Phenotypes in a Conditional Mouse Model of TDP-43 Proteinopathies

(2014) J. A. Alfieri et al.   JOURNAL OF NEUROSCIENCE

Intrinsic Membrane Hyperexcitability of Amyotrophic Lateral Sclerosis Patient-Derived Motor Neurons

(2014) Brian J. Wainger et al.   Cell Reports

Dipeptide repeat protein pathology in C9ORF72 mutation cases: clinico-pathological correlations

(2013) Ian R. Mackenzie et al.   ACTA NEUROPATHOLOGICA

Human von Economo Neurons Express Transcription Factors Associated with Layer V Subcerebral Projection Neurons

(2013) I. Cobos et al.   CEREBRAL CORTEX

Unconventional Translation of C9ORF72 GGGGCC Expansion Generates Insoluble Polypeptides Specific to c9FTD/ALS

(2013) Peter E.A. Ash et al.   NEURON

von Economo neurones are selectively targeted in frontotemporal dementia

(2013) A. F. Santillo et al.   NEUROPATHOLOGY AND APPLIED NEUROBIOLOGY

ALS-linked TDP-43 mutations produce aberrant RNA splicing and adult-onset motor neuron disease without aggregation or loss of nuclear TDP-43

(2013) E. S. Arnold et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

The C9orf72 GGGGCC Repeat Is Translated into Aggregating Dipeptide-Repeat Proteins in FTLD/ALS

(2013) K. Mori et al.   SCIENCE

Von Economo Neurons in the Anterior Insula of the Macaque Monkey

(2012) Henry C. Evrard et al.   NEURON

Role of right pregenual anterior cingulate cortex in self-conscious emotional reactivity

(2012) Virginia E. Sturm et al.   Social Cognitive and Affective Neuroscience

Clinical and neuropathologic heterogeneity of c9FTD/ALS associated with hexanucleotide repeat expansion in C9ORF72

(2011) Melissa E. Murray et al.   ACTA NEUROPATHOLOGICA

A harmonized classification system for FTLD-TDP pathology

(2011) Ian R. A. Mackenzie et al.   ACTA NEUROPATHOLOGICA

National Institute on Aging–Alzheimer’s Association guidelines for the neuropathologic assessment of Alzheimer’s disease: a practical approach

(2011) Thomas J. Montine et al.   ACTA NEUROPATHOLOGICA

Sensitivity of revised diagnostic criteria for the behavioural variant of frontotemporal dementia

(2011) Katya Rascovsky et al.   BRAIN

Distinctive Neurons of the Anterior Cingulate and Frontoinsular Cortex: A Historical Perspective

(2011) W. W. Seeley et al.   CEREBRAL CORTEX

Selective Frontoinsular von Economo Neuron and Fork Cell Loss in Early Behavioral Variant Frontotemporal Dementia

(2011) Eun-Joo Kim et al.   CEREBRAL CORTEX

Dysregulation of the ALS-associated gene TDP-43 leads to neuronal death and degeneration in mice

(2011) Lionel M. Igaz et al.   JOURNAL OF CLINICAL INVESTIGATION

Long pre-mRNA depletion and RNA missplicing contribute to neuronal vulnerability from loss of TDP-43

(2011) Magdalini Polymenidou et al.   NATURE NEUROSCIENCE

Expanded GGGGCC Hexanucleotide Repeat in Noncoding Region of C9ORF72 Causes Chromosome 9p-Linked FTD and ALS

(2011) Mariely DeJesus-Hernandez et al.   NEURON

A Hexanucleotide Repeat Expansion in C9ORF72 Is the Cause of Chromosome 9p21-Linked ALS-FTD

(2011) Alan E. Renton et al.   NEURON

Divergent network connectivity changes in behavioural variant frontotemporal dementia and Alzheimer’s disease

(2010) Juan Zhou et al.   BRAIN

Elevated expression of TDP-43 in the forebrain of mice is sufficient to cause neurological and pathological phenotypes mimicking FTLD-U

(2010) Kuen-Jer Tsai et al.   JOURNAL OF EXPERIMENTAL MEDICINE

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

Wild-Type Human TDP-43 Expression Causes TDP-43 Phosphorylation, Mitochondrial Aggregation, Motor Deficits, and Early Mortality in Transgenic Mice

(2010) Y.-F. Xu et al.   JOURNAL OF NEUROSCIENCE

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

(2010) Felix Geser et al.   NEUROPATHOLOGY

Sporadic corticobasal syndrome due to FTLD-TDP

(2009) Maria Carmela Tartaglia et al.   ACTA NEUROPATHOLOGICA

Nomenclature and nosology for neuropathologic subtypes of frontotemporal lobar degeneration: an update

(2009) Ian R. A. Mackenzie et al.   ACTA NEUROPATHOLOGICA

TDP-43 Redistribution is an Early Event in Sporadic Amyotrophic Lateral Sclerosis

(2009) Maria Teresa Giordana et al.   BRAIN PATHOLOGY

Neural basis of interpersonal traits in neurodegenerative diseases

(2009) Marc Sollberger et al.   NEUROPSYCHOLOGIA

Functional organization of the human anterior insular cortex

(2009) Isabella Mutschler et al.   NEUROSCIENCE LETTERS

TDP-43 mutant transgenic mice develop features of ALS and frontotemporal lobar degeneration

(2009) I. Wegorzewska et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Frontal Paralimbic Network Atrophy in Very Mild Behavioral Variant Frontotemporal Dementia

(2008) William W. Seeley et al.   ARCHIVES OF NEUROLOGY

Development of methodology for conducting clinical trials in frontotemporal lobar degeneration

(2008) D. S. Knopman et al.   BRAIN

Disturbance of Nuclear and Cytoplasmic TAR DNA-binding Protein (TDP-43) Induces Disease-like Redistribution, Sequestration, and Aggregate Formation

(2008) Matthew J. Winton et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Anterior horn cells with abnormal TDP-43 immunoreactivities show fragmentation of the Golgi apparatus in ALS

(2008) Yukio Fujita et al.   JOURNAL OF THE NEUROLOGICAL SCIENCES

How do you feel — now? The anterior insula and human awareness

(2008) A. D. (Bud) Craig   NATURE REVIEWS NEUROSCIENCE