Medium-Chain Fatty Acids Rescue Motor Function and Neuromuscular Junction Degeneration in a Drosophila Model of Amyotrophic Lateral Sclerosis (2023)
Journal Article
Dunn, E., Steinert, J. R., Stone, A., Sahota, V., Williams, R. S. B., Snowden, S., & Augustin, H. (2023). Medium-Chain Fatty Acids Rescue Motor Function and Neuromuscular Junction Degeneration in a Drosophila Model of Amyotrophic Lateral Sclerosis. Cells, 12(17), Article 2163. https://doi.org/10.3390/cells12172163

Amyotrophic lateral sclerosis (ALS) is an adult-onset neurodegenerative disease characterised by progressive degeneration of the motor neurones. An expanded GGGGCC (G4C2) hexanucleotide repeat in C9orf72 is the most common genetic cause of ALS and fr... Read More about Medium-Chain Fatty Acids Rescue Motor Function and Neuromuscular Junction Degeneration in a Drosophila Model of Amyotrophic Lateral Sclerosis.

The contribution of an imbalanced redox signalling to neurological and neurodegenerative conditions (2022)
Journal Article
Steinert, J. R., & Amal, H. (2023). The contribution of an imbalanced redox signalling to neurological and neurodegenerative conditions. Free Radical Biology and Medicine, 194, 71-83. https://doi.org/10.1016/j.freeradbiomed.2022.11.035

Nitric oxide and other redox active molecules such as oxygen free radicals provide essential signalling in diverse neuronal functions, but their excess production and insufficient scavenging induces cytotoxic redox stress which is associated with num... Read More about The contribution of an imbalanced redox signalling to neurological and neurodegenerative conditions.

Redox stress and metal dys-homeostasis appear as hallmarks of early prion disease pathogenesis in mice (2022)
Journal Article
Spiers, J. G., Cortina Chen, H. J., Barry, T. L., Bourgognon, J. M., & Steinert, J. R. (2022). Redox stress and metal dys-homeostasis appear as hallmarks of early prion disease pathogenesis in mice. Free Radical Biology and Medicine, 192, 182-190. https://doi.org/10.1016/j.freeradbiomed.2022.09.025

Neurodegenerative diseases are associated with a multitude of dysfunctional cellular pathways. One major contributory factor is a redox stress challenge during the development of several protein misfolding conditions including Alzheimer's (AD), Parki... Read More about Redox stress and metal dys-homeostasis appear as hallmarks of early prion disease pathogenesis in mice.

Kv3.3 subunits control presynaptic action potential waveform and neurotransmitter release at a central excitatory synapse (2022)
Journal Article
Richardson, A., Ciampani, V., Stancu, M., Bondarenko, K., Newton, S., Steinert, J. R., …Forsythe, I. D. (2022). Kv3.3 subunits control presynaptic action potential waveform and neurotransmitter release at a central excitatory synapse. eLife, 11, Article e75219. https://doi.org/10.7554/eLife.75219

Kv3 potassium currents mediate rapid repolarisation of action potentials (APs), supporting fast spikes and high repetition rates. Of the four Kv3 gene family members, Kv3.1 and Kv3.3 are highly expressed in the auditory brainstem and we exploited thi... Read More about Kv3.3 subunits control presynaptic action potential waveform and neurotransmitter release at a central excitatory synapse.

Author response: Kv3.3 subunits control presynaptic action potential waveform and neurotransmitter release at a central excitatory synapse (2022)
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Richardson, A., Ciampani, V., Stancu, M., Bondarenko, K., Newton, S., Steinert, J. R., …Forsythe, I. D. (2022). Author response: Kv3.3 subunits control presynaptic action potential waveform and neurotransmitter release at a central excitatory synapse. [elife]

Nitrergic modulation of neuronal excitability in the mouse hippocampus is mediated via regulation of Kv2 and voltage‐gated sodium channels (2021)
Journal Article
Scheiblich, H., & Steinert, J. R. (2021). Nitrergic modulation of neuronal excitability in the mouse hippocampus is mediated via regulation of Kv2 and voltage‐gated sodium channels. Hippocampus, 31(9), 1020-1038. https://doi.org/10.1002/hipo.23366

Regulation of neuronal activity is a necessity for communication and information transmission. Many regulatory processes which have been studied provide a complex picture of how neurons can respond to permanently changing functional requirements. One... Read More about Nitrergic modulation of neuronal excitability in the mouse hippocampus is mediated via regulation of Kv2 and voltage‐gated sodium channels.

Inhibition of neuroinflammatory nitric oxide signaling suppresses glycation and prevents neuronal dysfunction in mouse prion disease (2021)
Journal Article
Bourgognon, J., Spiers, J. G., Robinson, S. W., Scheiblich, H., Glynn, P., Ortori, C., …Steinert, J. R. (2021). Inhibition of neuroinflammatory nitric oxide signaling suppresses glycation and prevents neuronal dysfunction in mouse prion disease. Proceedings of the National Academy of Sciences, 118(10), Article e2009579118. https://doi.org/10.1073/pnas.2009579118

Several neurodegenerative diseases associated with protein misfolding (Alzheimer’s and Parkinson’s disease) exhibit oxidative and nitrergic stress following initiation of neuroinflammatory pathways. Associated nitric oxide (NO)-mediated posttranslati... Read More about Inhibition of neuroinflammatory nitric oxide signaling suppresses glycation and prevents neuronal dysfunction in mouse prion disease.

Nitrergic modulation of ion channel function in regulating neuronal excitability (2021)
Journal Article
Spiers, J. G., & Steinert, J. R. (2021). Nitrergic modulation of ion channel function in regulating neuronal excitability. Channels, 15(1), 666-679. https://doi.org/10.1080/19336950.2021.2002594

Nitric oxide (NO) signaling in the brain provides a wide range of functional properties in response to neuronal activity. NO exerts its effects through different signaling pathways, namely, through the canonical soluble guanylyl cyclase-mediated cGMP... Read More about Nitrergic modulation of ion channel function in regulating neuronal excitability.

The C terminus of p73 is essential for hippocampal development (2020)
Journal Article
Morone, N., Amelio, I., Panatta, E., Niklison-Chirou, M. V., Steinert, J. R., Agostini, M., …Knight, R. A. (2020). The C terminus of p73 is essential for hippocampal development. Proceedings of the National Academy of Sciences, 117(27), 15694-15701. https://doi.org/10.1073/pnas.2000917117

The p53 family member p73 has a complex gene structure, including alternative promoters and alternative splicing of the 3′ UTR. This results in a complex range of isoforms whose biological relevance largely remains to be determined. By deleting exon... Read More about The C terminus of p73 is essential for hippocampal development.

Kv3.1 and Kv3.3 subunits differentially contribute to Kv3 channels and action potential repolarization in principal neurons of the auditory brainstem (2020)
Journal Article
Choudhury, N., Linley, D., Richardson, A., Anderson, M., Robinson, S. W., Marra, V., …Forsythe, I. D. (2020). Kv3.1 and Kv3.3 subunits differentially contribute to Kv3 channels and action potential repolarization in principal neurons of the auditory brainstem. Journal of Physiology, 598(11), 2199-2222. https://doi.org/10.1113/jp279668

Kv3 voltage-gated potassium channels mediate action potential (AP) repolarization. The relative importance of Kv3.1 and Kv3.3 subunits for assembly of functional channels in neurons of the auditory brainstem was examined from the physiological perspe... Read More about Kv3.1 and Kv3.3 subunits differentially contribute to Kv3 channels and action potential repolarization in principal neurons of the auditory brainstem.