Results 41 to 50 of about 293,569 (356)

Potential of activated microglia as a source of dysregulated extracellular microRNAs contributing to neurodegeneration in amyotrophic lateral sclerosis [PDF]

, 2020
Amyotrophic lateral sclerosis (ALS) is the most common form of motor neuron degeneration in adults, and several mechanisms underlying the disease pathology have been proposed.
Christoforidou, Eleni, Hafezparast, Majid, Joilin, Greig   +2 more
core   +1 more source

Identification of pathways of degeneration and protection in motor neuron diseases [PDF]

, 2017
Motor neuron diseases preferentially affect specific neuronal populations with distinct clinical features even if disease-causing genes are expressed in many cell types. In spinal muscular atrophy (SMA), somatic motor neurons are selectively vulnerable
Nichterwitz, Susanne
core   +1 more source

Occasional essay: upper motor neuron syndrome in amyotrophic lateral sclerosis [PDF]

, 2020
The diagnosis of amyotrophic lateral sclerosis (ALS) requires recognition of both lower (LMN) and upper motor neuron (UMN) dysfunction.1 However, classical UMN signs are frequently difficult to identify in ALS.2 LMN involvement is sensitively detected ...
Burke, David, deCarvalho, Mamede, Grosskreutz, Julian, Kiernan, Matthew C, Leigh, P Nigel, Swash, Michael, Turner, Martin R   +6 more
core   +1 more source

The Role of DNA Damage in Neural Plasticity in Physiology and Neurodegeneration

Frontiers in Cellular Neuroscience, 2022
Damage to DNA is generally considered to be a harmful process associated with aging and aging-related disorders such as neurodegenerative diseases that involve the selective death of specific groups of neurons.
Anna Konopka, Julie D. Atkin, Julie D. Atkin   +2 more
doaj   +1 more source

Pulmonary Dysfunction Is Associated With Sleep Study Abnormalities in Children With Sickle Cell Disease: A Multicenter Study

Pediatric Blood &Cancer, EarlyView.
ABSTRACT Introduction Pulmonary dysfunction and sleep abnormalities are common in children with sickle cell disease (SCD) and are associated with worse clinical outcomes. Whether spirometry abnormalities are associated with polysomnography (PSG) findings remains unclear.
Ammar Saadoon Alishlash, Anis Rabbani Nourani, A. K. M. Fazlur Rahman, Dima Ezmigna, Tarig Ali‐Dinar   +4 more
wiley   +1 more source

Human TDP-43 overexpression in zebrafish motor neurons triggers MND-like phenotypes through gain-of-function mechanism

Acta Neuropathologica Communications
Dysregulation of the TAR DNA-binding protein 43 (TDP-43), including intraneuronal cytoplasmic mislocalisation and aggregation is a feature of multiple neurodegenerative diseases including amyotrophic lateral sclerosis (ALS), frontotemporal lobar dementia
Alison L. Hogan, Madison Kane, Patrick Chiu, Grant Richter, Cindy Maurel, Sharlynn Wu, Natalie M. Scherer, Emily K. Don, Albert Lee, Ian P. Blair, Roger S. Chung, Marco Morsch   +11 more
doaj   +1 more source

TDP-43 is a ubiquitylation substrate of the SCFcyclin F complex

Neurobiology of Disease, 2022
Background: Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease characterised by the loss of upper and lower motor neurons in the brain and spinal cord.
Stephanie L. Rayner, Shu Yang, Natalie E. Farrawell, Cyril J. Jagaraj, Flora Cheng, Jennilee M. Davidson, Luan Luu, Alberto G. Redondo, Alberto Rábano, Daniel Borrego-Hernández, Julie D. Atkin, Marco Morsch, Ian P. Blair, Justin J. Yerbury, Roger Chung, Albert Lee   +15 more
doaj   +1 more source

Comparative interactomics analysis of different ALS-associated proteins identifies converging molecular pathways [PDF]

, 2016
Amyotrophic lateral sclerosis (ALS) is a devastating neurological disease with no effective treatment available. An increasing number of genetic causes of ALS are being identified, but how these genetic defects lead to motor neuron degeneration and ...
Anink, Jasper J., Aronica, Eleonora, Blokhuis, Anna M., Bozzoni, Irene, Demmers, Jeroen A. A., Den Hertog, Jeroen, DINI MODIGLIANI, Stefano, Fumoto, Katsumi, Groen, Ewout J. N., Koppers, Max, Pasterkamp, R. Jeroen, Snelting, Anne, Sodaar, Peter, van Den Berg, Leonard H., van Den Heuvel, Dianne M. A., van Diggelen, Femke, Veldink, Jan H., Verheijen, Bert M.   +17 more
core   +3 more sources

Organoids in pediatric cancer research

FEBS Letters, EarlyView.
Organoid technology has revolutionized cancer research, yet its application in pediatric oncology remains limited. Recent advances have enabled the development of pediatric tumor organoids, offering new insights into disease biology, treatment response, and interactions with the tumor microenvironment.
Carla Ríos Arceo, Jarno Drost
wiley   +1 more source

Gain-of-function mutations in the ALS8 causative gene VAPB have detrimental effects on neurons and muscles

Biology Open, 2013
Summary Amyotrophic Lateral Sclerosis (ALS) is a motor neuron degenerative disease characterized by a progressive, and ultimately fatal, muscle paralysis. The human VAMP-Associated Protein B (hVAPB) is the causative gene of ALS type 8.
Mario Sanhueza, Luigi Zechini, Trudy Gillespie, Giuseppa Pennetta   +3 more
doaj   +1 more source