Results 271 to 280 of about 272,903 (354)

TARDBP (TDP‐43) Knock‐in Zebrafish Display a Late‐Onset Motor Phenotype and Loss of Large Spinal Cord Motor Neurons

open access: yesAnnals of Neurology, EarlyView.
Objective Mutations in TARDBP (encoding TDP‐43) are associated with the neurodegenerative disease amyotrophic lateral sclerosis (ALS) and include familial missense mutations where there are a lack of models and mechanisms examining how they are pathogenic.
Ziyaan A. Harji   +10 more
wiley   +1 more source

Resilience to Endoplasmic Reticulum Stress Mitigates Membrane Hyperexcitability Underlying Late Disease Onset in a Murine Model of SCA6

open access: yesAnnals of Neurology, EarlyView.
Objective An enduring puzzle in many inherited neurological disorders is the late onset of symptoms despite expression of function‐impairing mutant protein early in life. We examined the basis for onset of impairment in spinocerebellar ataxia type 6 (SCA6), a canonical late‐onset neurodegenerative ataxia which results from a polyglutamine expansion in ...
Haoran Huang   +10 more
wiley   +1 more source

Anatomical Progression of Neuropathology in FTLD‐TDP Type C and Linkage to Annexin A11

open access: yesAnnals of Neurology, EarlyView.
Objective Frontotemporal lobar degenerations (FTLD)‐TDP type C (TDP‐C) is distinguished from other FTLD‐TDP subtypes by 3 unique features: (1) invariable onset in the anterior temporal lobe (ATL), (2) phosphorylated TDP‐43 (pTDP) neurites in cortex, and (3) colocalization of all pTDP deposits with annexin A11 (ANXA11).
Allegra Kawles   +7 more
wiley   +1 more source

Expression of NR2B in Cerebellar Granule Cells Specifically Facilitates Effect of Motor Training on Motor Learning

open access: gold, 2008
Jianwei Jiao   +7 more
openalex   +2 more sources

Hydroxycarboxylic Acid Receptor 2 Mediates β‐hydroxybutyrate's Antiseizure Effect in Mice

open access: yesAnnals of Neurology, EarlyView.
A Objective The ketogenic diet, a high‐fat, low‐carbohydrate regimen, is often used to treat drug‐resistant seizures and is being studied for Alzheimer's disease and other neuropsychiatric disorders. However, its mechanism of action remains unclear. β‐hydroxybutyrate, a primary circulating ketone body produced by the ketogenic diet, may mediate its ...
Soudabeh Naderi   +10 more
wiley   +1 more source

Fibril Structure of Desiccation‐Protective Tardigrade Protein CAHS‐8

open access: yesAngewandte Chemie, EarlyView.
We present the atomic structure of the fibril formed by an intrinsically disordered protein that has been shown to be responsible for remarkable environmental stress in tardigrades. X‐ray crystallography reveals a unique mechanism of fibril assembly whereby a single helix forms a stable, antiparallel coiled‐coil dimer, which then assembles into a ...
Anas Malki   +6 more
wiley   +2 more sources

Generation of an enhancer-driven gene expression viral tool specific to dentate granule cell-types through direct hippocampal injection. [PDF]

open access: yesFront Neurosci
Potenza ML   +16 more
europepmc   +1 more source

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