The coming-of-age of nucleocytoplasmic transport in motor neuron disease and neurodegeneration
PA Ferreira - Cellular and Molecular Life Sciences, 2019 - Springer
The nuclear pore is the gatekeeper of nucleocytoplasmic transport and signaling through
which a vast flux of information is continuously exchanged between the nuclear and …
which a vast flux of information is continuously exchanged between the nuclear and …
[HTML][HTML] Glycine-alanine dipeptide repeats spread rapidly in a repeat length-and age-dependent manner in the fly brain
J Morón-Oset, T Supèr, J Esser, AM Isaacs… - Acta neuropathologica …, 2019 - Springer
Hexanucleotide repeat expansions of variable size in C9orf72 are the most prevalent
genetic cause of amyotrophic lateral sclerosis and frontotemporal dementia. Sense and …
genetic cause of amyotrophic lateral sclerosis and frontotemporal dementia. Sense and …
[HTML][HTML] Modeling C9orf72-Related Frontotemporal Dementia and Amyotrophic Lateral Sclerosis in Drosophila
An intronic hexanucleotide (GGGGCC) expansion in the C9orf72 gene is the most common
genetic cause of frontotemporal dementia (FTD) and amyotrophic lateral sclerosis (ALS). In …
genetic cause of frontotemporal dementia (FTD) and amyotrophic lateral sclerosis (ALS). In …
[HTML][HTML] Advances in the structure of GGGGCC repeat RNA sequence and its interaction with small molecules and protein partners
X Liu, X Zhao, J He, S Wang, X Shen, Q Liu, S Wang - Molecules, 2023 - mdpi.com
The aberrant expansion of GGGGCC hexanucleotide repeats within the first intron of the
C9orf72 gene represent the predominant genetic etiology underlying amyotrophic lateral …
C9orf72 gene represent the predominant genetic etiology underlying amyotrophic lateral …
The prion-like nature of amyotrophic lateral sclerosis
The misfolding, aggregation, and deposition of specific proteins is the key hallmark of most
progressive neurodegenerative disorders such as Alzheimer's disease, Parkinson's disease …
progressive neurodegenerative disorders such as Alzheimer's disease, Parkinson's disease …
Exploring the alternative: fish, flies and worms as preclinical models for ALS
E Braems, P Tziortzouda, L Van Den Bosch - Neuroscience Letters, 2021 - Elsevier
Amyotrophic lateral sclerosis (ALS) is an incurable neurodegenerative disorder
characterized by the loss of upper and lower motor neurons. In general, patients succumb to …
characterized by the loss of upper and lower motor neurons. In general, patients succumb to …
[HTML][HTML] Measuring and interpreting nuclear transport in neurodegenerative disease—The example of C9orf72 ALS
MFW Semmelink, A Steen, LM Veenhoff - International Journal of …, 2021 - mdpi.com
Transport from and into the nucleus is essential to all eukaryotic life and occurs through the
nuclear pore complex (NPC). There are a multitude of data supporting a role for nuclear …
nuclear pore complex (NPC). There are a multitude of data supporting a role for nuclear …
Presynaptic accumulation of α-synuclein causes synaptopathy and progressive neurodegeneration in Drosophila
JC Bridi, E Bereczki, SK Smith, GM Poças… - Brain …, 2021 - academic.oup.com
Abstract Alpha-synuclein (α-syn) mislocalization and accumulation in intracellular inclusions
is the major pathological hallmark of degenerative synucleinopathies, including Parkinson's …
is the major pathological hallmark of degenerative synucleinopathies, including Parkinson's …
Modelling the pathology and behaviour of frontotemporal dementia
DA Solomon, JC Mitchell… - Neuropathology and …, 2019 - Wiley Online Library
Frontotemporal dementia (FTD) encompasses a collection of clinically and pathologically
diverse neurological disorders. Clinical features of behavioural and language dysfunction …
diverse neurological disorders. Clinical features of behavioural and language dysfunction …
[HTML][HTML] Disease Mechanisms and Therapeutic Approaches in C9orf72 ALS-FTD
A hexanucleotide repeat expansion mutation in the first intron of C9orf72 is the most
common known genetic cause of amyotrophic lateral sclerosis and frontotemporal dementia …
common known genetic cause of amyotrophic lateral sclerosis and frontotemporal dementia …