Isabel Hubbard

Glial cell toxicity in a Drosophila C9orf72 neurodegeneration model

Isabel Hubbard, Josh Dubnau

The most common genetic cause of both familial amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) is an expanded G4C2 repeat in the first intron of the gene C9orf72. The C9orf72 repeat expansion is bidirectionally transcribed into sense and anti-sense RNA foci, …

us (code pays fourni par la source)

Glial toxicity in a Drosophila C9orf72 neurodegeneration model

Isabel Hubbard, Josh Dubnau

The most common genetic cause of both familial amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) is an expanded G4C2 repeat in the first intron of the gene C9orf72. The C9orf72 repeat expansion is bidirectionally transcribed into sense and anti-sense RNA foci, …

us (code pays fourni par la source)

Glial cell-intrinsic and non-cell autonomous toxicity in a Drosophila C9orf72 neurodegeneration model

Isabel Hubbard, Josh Dubnau

Abstract The most common genetic cause of both familial amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) is an expanded G 4 C 2 repeat in the first intron of the gene C9orf72. The C9orf72 repeat expansion is bidirectionally transcribed into sense …

us (code pays fourni par la source)

TDP-43 pathology in Drosophila induces glial-cell type specific toxicity that can be ameliorated by knock-down of SF2/SRSF1

Sarah Krupp, Isabel Hubbard, Oliver H. Tam, Molly Hammell et autres

Accumulation of cytoplasmic inclusions of TAR-DNA binding protein 43 (TDP-43) is seen in both neurons and glia in a range of neurodegenerative disorders, including amyotrophic lateral sclerosis (ALS), frontotemporal dementia (FTD) and Alzheimer's disease (AD). Disease progression involves non-cell autonomous interactions among …

us (code pays fourni par la source)

International Practice Recommendations for the Recognition and Management of Hearing and Vision Impairment in People with Dementia

Jenna Louise Littlejohn, Michael A. Bowen, Fofi Constantinidou, Piers Dawes et autres

INTRODUCTION: Hearing, vision, and cognitive impairment commonly co-occur in older people. However, the rate of recognition and appropriate management of combined hearing and vision impairment in people with dementia impairment is low. The aim of this work was to codevelop internationally relevant, …

gb, cy, au, us, ca, ie (code pays fourni par la source)

Truncated stathmin-2 is a marker of TDP-43 pathology in frontotemporal dementia

Mercedes Prudencio, Jack Humphrey, Sarah R. Pickles, Anna‐Leigh Brown et autres

No treatment for frontotemporal dementia (FTD), the second most common type of early-onset dementia, is available, but therapeutics are being investigated to target the 2 main proteins associated with FTD pathological subtypes: TDP-43 (FTLD-TDP) and tau (FTLD-tau). Testing potential therapies in clinical …

us, gb (code pays fourni par la source)

Postmortem Cortex Samples Identify Distinct Molecular Subtypes of ALS: Retrotransposon Activation, Oxidative Stress, and Activated Glia

Oliver H. Tam, Nikolay V. Rozhkov, Regina Shaw, Duyang Kim et autres

Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease characterized by the progressive loss of motor neurons. While several pathogenic mutations have been identified, the vast majority of ALS cases have no family history of disease. Thus, for most ALS cases, the …

us (code pays fourni par la source)

Postmortem Cortex Samples Identify Distinct Molecular Subtypes of ALS: Retrotransposon Activation, Oxidative Stress, and Activated Glia

Oliver H. Tam, Nikolay V. Rozhkov, Regina Shaw, Duyang Kim et autres

Summary Amyotrophic Lateral Sclerosis (ALS) is a fatal neurodegenerative disease characterized by the progressive loss of motor neurons. While several inherited pathogenic mutations have been identified as causative, the vast majority of cases are sporadic with no family history of disease. Thus, …

us (code pays fourni par la source)

Postmortem Cortex Samples Identify Distinct Molecular Subtypes of ALS: Retrotransposon Activation, Oxidative Stress, and Activated Glia

Oliver H. Tam, Nikolay V. Rozhkov, Regina Shaw, Duyang Kim et autres

us (code pays fourni par la source)

Unexpected similarities between C9ORF72 and sporadic forms of ALS/FTD suggest a common disease mechanism

Erin G. Conlon, Delphine Fagegaltier, Phaedra Agius, Julia Davis‐Porada et autres

Amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) represent two ends of a disease spectrum with shared clinical, genetic and pathological features. These include near ubiquitous pathological inclusions of the RNA-binding protein (RBP) TDP-43, and often the presence of a GGGGCC expansion …

us, nl, gb, gr, il (code pays fourni par la source)

Author response: Unexpected similarities between C9ORF72 and sporadic forms of ALS/FTD suggest a common disease mechanism

Erin G. Conlon, Delphine Fagegaltier, Phaedra Agius, Julia Davis‐Porada et autres

us, mx, nl, gb, gr, il (code pays fourni par la source)

Rates of Amyloid Imaging Positivity in Patients With Primary Progressive Aphasia

Miguel Santos‐Santos, Gil D. Rabinovici, Leonardo Iaccarino, Nagehan Ayakta et autres

Importance: The ability to predict the pathology underlying different neurodegenerative syndromes is of critical importance owing to the advent of molecule-specific therapies. Objective: To determine the rates of positron emission tomography (PET) amyloid positivity in the main clinical variants of primary progressive …

es, us, it, kr (code pays fourni par la source)