[HTML][HTML] Seizing the moment: Zebrafish epilepsy models

K Gawel, M Langlois, T Martins, W van der Ent… - Neuroscience & …, 2020 - Elsevier
Zebrafish are now widely accepted as a valuable animal model for a number of different
central nervous system (CNS) diseases. They are suitable both for elucidating the origin of …

[HTML][HTML] Functional and biochemical consequences of disease variants in neurotransmitter transporters: a special emphasis on folding and trafficking deficits

S Bhat, A El-Kasaby, M Freissmuth, S Sucic - Pharmacology & therapeutics, 2021 - Elsevier
Neurotransmitters, such as γ-aminobutyric acid, glutamate, acetyl choline, glycine and the
monoamines, facilitate the crosstalk within the central nervous system. The designated …

Stress response silencing by an E3 ligase mutated in neurodegeneration

DL Haakonsen, M Heider, AJ Ingersoll, K Vodehnal… - Nature, 2024 - nature.com
Stress response pathways detect and alleviate adverse conditions to safeguard cell and
tissue homeostasis, yet their prolonged activation induces apoptosis and disrupts …

UBE2A and UBE2B are recruited by an atypical E3 ligase module in UBR4

L Barnsby-Greer, PD Mabbitt, MA Dery… - Nature Structural & …, 2024 - nature.com
UBR4 is a 574 kDa E3 ligase (E3) of the N-degron pathway with roles in neurodevelopment,
age-associated muscular atrophy and cancer. The catalytic module that carries out ubiquitin …

[HTML][HTML] Episodic ataxias: Primary and secondary etiologies, treatment, and classification approaches

A Hassan - Tremor and Other Hyperkinetic Movements, 2023 - ncbi.nlm.nih.gov
Background: Episodic ataxia (EA), characterized by recurrent attacks of cerebellar
dysfunction, is the manifestation of a group of rare autosomal dominant inherited disorders …

Clinical and genetic overview of paroxysmal movement disorders and episodic ataxias

G Garone, A Capuano, L Travaglini, F Graziola… - International Journal of …, 2020 - mdpi.com
Paroxysmal movement disorders (PMDs) are rare neurological diseases typically
manifesting with intermittent attacks of abnormal involuntary movements. Two main …

Molecular insights into disease-associated glutamate transporter (EAAT1 / SLC1A3) variants using in silico and in vitro approaches

M Gorostiola González, HJ Sijben… - Frontiers in Molecular …, 2023 - frontiersin.org
Glutamate is an essential excitatory neurotransmitter and an intermediate for energy
metabolism. Depending on the tumor site, cancer cells have increased or decreased …

Episodic ataxias

JC Jen, J Wan - Handbook of clinical neurology, 2018 - Elsevier
Primary episodic ataxias (EAs) are a group of dominantly inherited disorders characterized
by transient recurrent incoordination and truncal instability, often triggered by physical …

Cellular physiology and pathophysiology of EAAT anion channels

P Kovermann, M Engels, F Müller… - Frontiers in cellular …, 2022 - frontiersin.org
Excitatory amino acid transporters (EAATs) optimize the temporal resolution and energy
demand of mammalian excitatory synapses by quickly removing glutamate from the synaptic …

[HTML][HTML] Abnormal eye movements in parkinsonism and movement disorders

I Jung, JS Kim - Journal of movement disorders, 2019 - ncbi.nlm.nih.gov
Abnormal eye movements are commonly observed in movement disorders. Ocular motility
examination should include bedside evaluation and laboratory recording of ocular …