Results 91 to 100 of about 164,738 (314)

Mutant GGGGCC RNA prevents YY1 from binding to Fuzzy promoter which stimulates Wnt/β-catenin pathway in C9ALS/FTD

Nature Communications, 2023
The GGGGCC hexanucleotide repeat expansion mutation in the chromosome 9 open reading frame 72 (C9orf72) gene is a major genetic cause of amyotrophic lateral sclerosis and frontotemporal dementia (C9ALS/FTD).
Zhefan Stephen Chen, Mingxi Ou, Stephanie Taylor, Ruxandra Dafinca, Shaohong Isaac Peng, Kevin Talbot, Ho Yin Edwin Chan   +6 more
doaj   +1 more source

Advances in motor neurone disease [PDF]

Journal of the Royal Society of Medicine, 2014
Summary Motor neurone disease (MND), the commonest clinical presentation of which is amyotrophic lateral sclerosis (ALS), is regarded as the most devastating of adult-onset neurodegenerative disorders. The last decade has seen major improvements in patient care, but also rapid scientific advances, so that rational therapies based on ...
Bäumer, D, Talbot, K, Turner, M
openaire   +2 more sources

Phosphoinositides and inositol phosphates as molecular glues

FEBS Letters, EarlyView.
Inositol phosphates (IPs) and phosphoinositides (PIPs) regulate diverse eukaryotic processes. Beyond recruiting signaling proteins or acting as structural cofactors, recent studies suggest they mediate protein–protein interactions as natural molecular glues.
Aleshia Seaton‐Terry, Alexander J. Cutright, Mong Na Claire Loi, Jessica Martin, Joseph W. Gilligan, Jakub N. Kubina, Michael J. Arambula, Susanna Tanada Palmu, Daniella Zhou, Raymond D. Blind   +9 more
wiley   +1 more source

Gut transit and gut microbiome changes occur prior to the onset of motor impairment in a mouse model of Machado-Joseph disease

Neurobiology of Disease
We previously identified microbial shifts prior to the onset of motor and neurological symptoms within a mouse model of the fatal neurodegenerative disease Machado-Joseph disease (MJD).
Vasilisa Zvyagina, Andrea Kuriakose, Ignacio Simó, Julia Y. Kam, Prapti Chakraborty, Katherine J. Robinson, Anastasiya Potapenko, Maxinne Watchon, Ian T. Paulsen, Hasinika K.A.H. Gamage, Angela S. Laird   +10 more
doaj   +1 more source

Neuronal cell culture from transgenic zebrafish models of neurodegenerative disease

Biology Open, 2018
We describe a protocol for culturing neurons from transgenic zebrafish embryos to investigate the subcellular distribution and protein aggregation status of neurodegenerative disease-causing proteins.
Jamie R. Acosta, Maxinne Watchon, Kristy C. Yuan, Jennifer A. Fifita, Adam J. Svahn, Emily K. Don, Claire G. Winnick, Ian P. Blair, Garth A. Nicholson, Nicholas J. Cole, Claire Goldsbury, Angela S. Laird   +11 more
doaj   +1 more source

Assessing motor neuron disease [PDF]

QJM, 2002
Sir, In their paper ( Q J Med 2001; 94 :491–5), Hadjikoutis and Wiles draw attention to a number of circumstances in which the serum chloride and bicarbonate can be …
openaire   +2 more sources

PARK(ing) time–How park deficiency affects the biological clock in a Drosophila model of Parkinson's disease

FEBS Letters, EarlyView.
Drosophila park mutants serve as a model for Parkinson's disease. We used this strain to investigate the connection between oxidative stress and the circadian clock mechanism. We showed that increased oxidative stress affects the physiology of pacemaker cells, disrupting their daily structural plasticity. Lack of rhythmic signaling from pacemaker cells
Kamila Zientara, Kinga Skoczek, Elzbieta Pyza, Milena Damulewicz   +3 more
wiley   +1 more source

Loss-of-function variant in the ovine TMCO6 gene in north country Cheviot sheep with motor neuron disease.

, 2023
In North Country Cheviot lambs with early-onset progressive ataxia and motor neuron degeneration, whole-genome sequencing identified a homozygous loss-of-function variant in the ovine transmembrane and coiled-coil domains (TMCO6) gene.
Brülisauer, Franz, Häfliger, Irene M, Franz Brülisauer, Sandra Scholes, Drögemüller, Cord, Letko, Anna, Irene M. Häfliger, Anna Letko, Corr, Eilidh, Scholes, Sandra, Cord Drögemüller, Eilidh Corr   +11 more
core   +1 more source

Proteomics Approaches for Biomarker and Drug Target Discovery in ALS and FTD

Frontiers in Neuroscience, 2019
Neurodegenerative disorders such as amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) are increasing in prevalence but lack targeted therapeutics. Although the pathological mechanisms behind these diseases remain unclear, both ALS and
Thomas J. Hedl, Rebecca San Gil, Flora Cheng, Stephanie L. Rayner, Jennilee M. Davidson, Alana De Luca, Maria D. Villalva, Heath Ecroyd, Heath Ecroyd, Adam K. Walker, Adam K. Walker, Albert Lee   +11 more
doaj   +1 more source

Tumour–host interactions in Drosophila: mechanisms in the tumour micro‐ and macroenvironment

Molecular Oncology, EarlyView.
This review examines how tumour–host crosstalk takes place at multiple levels of biological organisation, from local cell competition and immune crosstalk to organism‐wide metabolic and physiological collapse. Here, we integrate findings from Drosophila melanogaster studies that reveal conserved mechanisms through which tumours hijack host systems to ...
José Teles‐Reis, Tor Erik Rusten
wiley   +1 more source