Early Cognitive/Social Deficits and Late Motor Phenotype in Conditional Wild-Type TDP-43 Transgenic Mice

A transgenic mouse expressing CHMP2Bintron5mutant in neurons develops histological and behavioural features of amyotrophic lateral sclerosis and frontotemporal dementia

(2016) Aurélia Vernay et al.   HUMAN MOLECULAR GENETICS

The clinical spectrum of sporadic and familial forms of frontotemporal dementia

(2016) Ione O. C. Woollacott et al.   JOURNAL OF NEUROCHEMISTRY

Hyperactive somatostatin interneurons contribute to excitotoxicity in neurodegenerative disorders

(2016) Wen Zhang et al.   NATURE NEUROSCIENCE

Mutant Tau knock-in mice display frontotemporal dementia relevant behaviour and histopathology

(2016) David J. Koss et al.   NEUROBIOLOGY OF DISEASE

C9orf72 BAC Mouse Model with Motor Deficits and Neurodegenerative Features of ALS/FTD

(2016) Yuanjing Liu et al.   NEURON

Disinhibition-like behavior in a P301S mutant tau transgenic mouse model of frontotemporal dementia

(2016) Magdalena Przybyla et al.   NEUROSCIENCE LETTERS

Progression of motor neuron disease is accelerated and the ability to recover is compromised with advanced age in rNLS8 mice

(2016) Krista J. Spiller et al.   Acta Neuropathologica Communications

From animal models to human disease: a genetic approach for personalized medicine in ALS

(2016) Vincent Picher-Martel et al.   Acta Neuropathologica Communications

Short-term suppression of A315T mutant human TDP-43 expression improves functional deficits in a novel inducible transgenic mouse model of FTLD-TDP and ALS

(2015) Yazi D. Ke et al.   ACTA NEUROPATHOLOGICA

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

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

Behavioral phenotypes of genetic mouse models of autism

(2015) T. M. Kazdoba et al.   GENES BRAIN AND BEHAVIOR

Evaluating Behavior in Mouse Models of the Behavioral Variant of Frontotemporal Dementia: Which Test for Which Symptom?

(2015) Aurélia Vernay et al.   Neurodegenerative Diseases

Altered Corticostriatal Connectivity and Exploration/Exploitation Imbalance Emerge as Intermediate Phenotypes for a Neonatal Dopamine Dysfunction

(2015) Barbara Y Braz et al.   NEUROPSYCHOPHARMACOLOGY

The role of TDP-43 in the pathogenesis of ALS and FTLD: Figure 1

(2015) Marco Baralle et al.   BIOCHEMICAL SOCIETY TRANSACTIONS

Transcriptomic Changes Due to Cytoplasmic TDP-43 Expression Reveal Dysregulation of Histone Transcripts and Nuclear Chromatin

(2015) Alexandre Amlie-Wolf et al.   PLoS One

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

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

Alterations in microRNA-124 and AMPA receptors contribute to social behavioral deficits in frontotemporal dementia

(2014) Eduardo Gascon et al.   NATURE MEDICINE

Axonal Transport of TDP-43 mRNA Granules Is Impaired by ALS-Causing Mutations

(2014) Nael H. Alami et al.   NEURON

Behavioral and language variants of frontotemporal dementia: A review of key symptoms

(2013) Robert Laforce   CLINICAL NEUROLOGY AND NEUROSURGERY

Disease Animal Models of TDP-43 Proteinopathy and Their Pre-Clinical Applications

(2013) Yu-Chih Liu et al.   INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES

Dissociation of Frontotemporal Dementia-Related Deficits and Neuroinflammation in Progranulin Haploinsufficient Mice

(2013) A. J. Filiano et al.   JOURNAL OF NEUROSCIENCE

Amyotrophic Lateral Sclerosis: An update for 2013 Clinical Features, Pathophysiology, Management and Therapeutic Trials

(2013) Paul Gordon et al.   Aging and Disease

TDP-43 regulates the mammalian spinogenesis through translational repression of Rac1

(2012) Pritha Majumder et al.   ACTA NEUROPATHOLOGICA

Mouse models of frontotemporal dementia

(2012) Erik D. Roberson   ANNALS OF NEUROLOGY

Rodent models of TDP-43: Recent advances

(2012) William Tsao et al.   BRAIN RESEARCH

Frontotemporal Lobar Degeneration

(2012) Rachel E. Seltman et al.   CNS DRUGS

FTD and ALS: A Tale of Two Diseases

(2011) R. Ferrari et al.   Current Alzheimer Research

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

TDP-43 Autoregulation: Implications for Disease

(2011) Mauricio Budini et al.   JOURNAL OF MOLECULAR NEUROSCIENCE

Core features of frontotemporal dementia recapitulated in progranulin knockout mice

(2011) N. Ghoshal et al.   NEUROBIOLOGY OF DISEASE

P301S Mutant Human Tau Transgenic Mice Manifest Early Symptoms of Human Tauopathies with Dementia and Altered Sensorimotor Gating

(2011) Hiroki Takeuchi et al.   PLoS One

TDP-43 regulates its mRNA levels through a negative feedback loop

(2010) Youhna M Ayala et al.   EMBO JOURNAL

Behavioral deficits and progressive neuropathology in progranulin-deficient mice: a mouse model of frontotemporal dementia

(2010) Fangfang Yin et al.   FASEB JOURNAL

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

Dementia and cognitive impairment in amyotrophic lateral sclerosis: a review

(2010) Maria Teresa Giordana et al.   NEUROLOGICAL SCIENCES

TDP-43 Depletion Induces Neuronal Cell Damage through Dysregulation of Rho Family GTPases

(2009) Yohei Iguchi et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Postnatal NMDA receptor ablation in corticolimbic interneurons confers schizophrenia-like phenotypes

(2009) Juan E Belforte et al.   NATURE NEUROSCIENCE

Findings from animals concerning when interactions between perirhinal cortex, hippocampus and medial prefrontal cortex are necessary for recognition memory

(2009) E. Clea Warburton et al.   NEUROPSYCHOLOGIA

TDP-43, the signature protein of FTLD-U, is a neuronal activity-responsive factor

(2008) I.-Fan Wang et al.   JOURNAL OF NEUROCHEMISTRY