Results 71 to 80 of about 293,569 (356)

Degeneration and impaired regeneration of gray matter oligodendrocytes in amyotrophic lateral sclerosis. [PDF]

, 2013
Oligodendrocytes associate with axons to establish myelin and provide metabolic support to neurons. In the spinal cord of amyotrophic lateral sclerosis (ALS) mice, oligodendrocytes downregulate transporters that transfer glycolytic substrates to neurons ...
Bergles, Dwight E, Cleveland, Don W, Fukaya, Masahiro, Kang, Shin H, Li, Ying, Lorenzini, Ileana, Ostrow, Lyle W, Rothstein, Jeffrey D   +7 more
core   +2 more sources

Valosin‐containing protein counteracts ATP‐driven dissolution of FUS condensates through its ATPase activity in vitro

FEBS Letters, EarlyView.
Biomolecular condensates formed by fused in sarcoma (FUS) are dissolved by high ATP concentrations yet persist in cells. Using a reconstituted system, we demonstrate that valosin‐containing protein (VCP), an AAA+ ATPase, counteracts ATP‐driven dissolution of FUS condensates through its D2 ATPase activity.
Hitomi Kimura, Shin‐ichi Tate, Kyota Yasuda   +2 more
wiley   +1 more source

Advances in nanotechnology-based strategies for the treatments of amyotrophic lateral sclerosis

Materials Today Bio, 2020
Amyotrophic lateral sclerosis (ALS), also known as motor neuron disease (MND), is a progressive neurodegenerative disease that affects both upper and lower motor neurons, which results in loss of muscle control and eventual paralysis [1].
G.Y. Wang, S.L. Rayner, R. Chung, B.Y. Shi, X.J. Liang   +4 more
doaj   +1 more source

Diversity and complexity in neural organoids

FEBS Letters, EarlyView.
Neural organoid research aims to expand genetic diversity on one side and increase tissue complexity on the other. Chimeroids integrate multiple donor genomes within single organoids. Self‐organising multi‐identity organoids, exogenous cell seeding, or enforced assembly of region‐specific organoids contribute to tissue complexity.
Ilaria Chiaradia, Madeline A. Lancaster
wiley   +1 more source

Multi-region brain transcriptomic analysis of amyotrophic lateral sclerosis reveals widespread RNA alterations and substantial cerebellum involvement

Molecular Neurodegeneration
Background Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease that primarily affects the motor neurons, causing progressive muscle weakness and paralysis.
Natalie Grima, Andrew N. Smith, Claire E. Shepherd, Lyndal Henden, Thiri Zaw, Luke Carroll, Dominic B. Rowe, Matthew C. Kiernan, Ian P. Blair, Kelly L. Williams   +9 more
doaj   +1 more source

Glial Cells in Amyotrophic Lateral Sclerosis

Neurology Research International, 2011
Amyotrophic lateral sclerosis (ALS) is an adult motor neuron disease characterized by premature death of upper and lower motor neurons. Two percent of ALS cases are caused by the dominant mutations in the gene for superoxide dismutase 1 (SOD1) through a ...
Jurate Lasiene, Koji Yamanaka
doaj   +1 more source

Linking neurogenesis, oligodendrogenesis, and myelination defects to neurodevelopmental disruption in primary mitochondrial disorders

FEBS Letters, EarlyView.
Mitochondrial remodeling shapes neural and glial lineage progression by matching metabolic supply with demand. Elevated OXPHOS supports differentiation and myelin formation, while myelin compaction lowers mitochondrial dependence, revealing mitochondria as key drivers of developmental energy adaptation.
Sahitya Ranjan Biswas, Porter L. Tomsick, Alicia M. Pickrell, Paul D. Morton   +3 more
wiley   +1 more source

Dysfunction of Optineurin in Amyotrophic Lateral Sclerosis and Glaucoma

Frontiers in Immunology, 2018
Neurodegenerative disorders, including amyotrophic lateral sclerosis (ALS), frontotemporal dementia, and glaucoma, affect millions of people worldwide.
Reka P. Toth, Julie D. Atkin, Julie D. Atkin   +2 more
doaj   +1 more source

Early exposure to environmental toxin contributes to neuronal vulnerability and axonal pathology in a model of familial ALS [PDF]

, 2011
Adult onset amyotrophic lateral sclerosis (ALS) arises due to progressive and irreversible functional deficits to the central nervous system, specifically the loss of motor neurons.
Christopher A. Shaw, Grace Lee
core   +1 more source

Single copy/knock-in models of ALS SOD1 in C. elegans suggest loss and gain of function have different contributions to cholinergic and glutamatergic neurodegeneration [PDF]

, 2018
Mutations in Cu/Zn superoxide dismutase 1 (SOD1) lead to Amyotrophic Lateral Sclerosis (ALS), a neurodegenerative disease that disproportionately affects glutamatergic and cholinergic motor neurons.
Baskoylu, Saba N, Dimitriadi, Maria, Grosser, Sarah, Hart, Anne C, Kim, Sarah, Lins, Jeremy, O'Hern, Patrick, Schuch, Kelsey, Simon, Jonah, Yanagi, Katherine S, Yersak, Jill   +10 more
core   +3 more sources