Inflammation in ALS/FTD pathogenesis

ME McCauley, RH Baloh - Acta neuropathologica, 2019 - Springer
Amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) are
neurodegenerative diseases that overlap in their clinical presentation, pathology and …

Emerging mechanisms of molecular pathology in ALS

OM Peters, M Ghasemi… - The Journal of clinical …, 2015 - Am Soc Clin Investig
Amyotrophic lateral sclerosis (ALS) is a devastating degenerative disease characterized by
progressive loss of motor neurons in the motor cortex, brainstem, and spinal cord. Although …

CRISPR–Cas9 screens in human cells and primary neurons identify modifiers of C9ORF72 dipeptide-repeat-protein toxicity

NJ Kramer, MS Haney, DW Morgens, A Jovičić… - Nature …, 2018 - nature.com
Hexanucleotide-repeat expansions in the C9ORF72 gene are the most common cause of
amyotrophic lateral sclerosis and frontotemporal dementia (c9ALS/FTD). The nucleotide …

Identification of evolutionarily conserved gene networks mediating neurodegenerative dementia

V Swarup, FI Hinz, JE Rexach, K Noguchi… - Nature medicine, 2019 - nature.com
Identifying the mechanisms through which genetic risk causes dementia is an imperative for
new therapeutic development. Here, we apply a multistage, systems biology approach to …

The clinical spectrum of sporadic and familial forms of frontotemporal dementia

IOC Woollacott, JD Rohrer - Journal of neurochemistry, 2016 - Wiley Online Library
The term frontotemporal dementia (FTD) describes a clinically, genetically and
pathologically diverse group of neurodegenerative disorders. Symptoms of FTD can present …

Innate immunity: a common denominator between neurodegenerative and neuropsychiatric diseases

F Novellino, V Saccà, A Donato, P Zaffino… - International journal of …, 2020 - mdpi.com
The intricate relationships between innate immunity and brain diseases raise increased
interest across the wide spectrum of neurodegenerative and neuropsychiatric disorders …

Genetics of FTLD: overview and what else we can expect from genetic studies

C Pottier, TA Ravenscroft… - Journal of …, 2016 - Wiley Online Library
Frontotemporal lobar degeneration (FTLD) comprises a highly heterogeneous group of
disorders clinically associated with behavioral and personality changes, language …

Implementation of an antibody characterization procedure and application to the major ALS/FTD disease gene C9ORF72

C Laflamme, PM McKeever, R Kumar, J Schwartz… - Elife, 2019 - elifesciences.org
Antibodies are a key resource in biomedical research yet there are no community-accepted
standards to rigorously characterize their quality. Here we develop a procedure to validate …

TDP-43 real-time quaking induced conversion reaction optimization and detection of seeding activity in CSF of amyotrophic lateral sclerosis and frontotemporal …

C Scialò, TH Tran, G Salzano, G Novi… - Brain …, 2020 - academic.oup.com
The pathological deposition of the transactive response DNA-binding protein of 43 kDa
occurs in the majority (∼ 97%) of amyotrophic lateral sclerosis and in around 45% of …

Different neuroinflammatory profile in amyotrophic lateral sclerosis and frontotemporal dementia is linked to the clinical phase

P Oeckl, P Weydt, P Steinacker… - Journal of Neurology …, 2019 - jnnp.bmj.com
Objective To investigate the role of neuroinflammation in asymptomatic and symptomatic
amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) mutation carriers …