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All Outputs (23)

The contribution of multicellular model organisms to Neuronal Ceroid Lipofuscinosis research (2019)
Journal Article
Russell, C., Huber, R., Hughes, S., Wenfei Liu, Morgan, A., & Tuxworth, R. (2019). The contribution of multicellular model organisms to Neuronal Ceroid Lipofuscinosis research. https://doi.org/10.1016/j.bbadis.2019.165614

The NCLs (neuronal ceroid lipofuscinosis) are forms of neurodegenerative disease that affect people of all ages and ethnicities but are most prevalent in children. Commonly known as Batten disease, this debilitating neurological disorder is comprised... Read More about The contribution of multicellular model organisms to Neuronal Ceroid Lipofuscinosis research.

Zebrafish as a model for kidney function and disease (2019)
Journal Article
Outtandy, P., Russell, C., Kleta, R., & Brockenhauer, D. (2019). Zebrafish as a model for kidney function and disease. Pediatric Nephrology, 34(5), 751-762. https://doi.org/10.1007/s00467-018-3921-7

Kidney disease is a global problem with around three million people diagnosed in the UK alone and the incidence is rising. Research is critical to develop better treatments. Animal models can help to better understand the pathophysiology behind the v... Read More about Zebrafish as a model for kidney function and disease.

Reduction of the ATPase inhibitory factor 1 (IF1) leads to visual impairment in vertebrates (2018)
Journal Article
Martin-Jimenez, R., Faccenda, D., Allen, E., Reichel, H. B., Arcos, L., Ferraina, C., Strobbe, D., Russell, C., & Campanella, M. (2018). Reduction of the ATPase inhibitory factor 1 (IF1) leads to visual impairment in vertebrates. https://doi.org/10.1038/s41419-018-0578-x

In vertebrates, mitochondria are tightly preserved energy producing organelles, which sustain nervous system development and function. The understanding of proteins that regulate their homoeostasis in complex animals is therefore critical and doing s... Read More about Reduction of the ATPase inhibitory factor 1 (IF1) leads to visual impairment in vertebrates.

Neurodegeneration and Epilepsy in a Zebrafish Model of CLN3 Disease (Batten Disease) (2016)
Journal Article
Wager, K., Zdebik, A. A., Fu, S., Cooper, J. D., Harvey, R. J., & Russell, C. (2016). Neurodegeneration and Epilepsy in a Zebrafish Model of CLN3 Disease (Batten Disease). PLoS ONE, 11(6), https://doi.org/10.1371/journal.pone.0157365

The neuronal ceroid lipofuscinoses are a group of lysosomal storage disorders that comprise the most common, genetically heterogeneous, fatal neurodegenerative disorders of children. They are characterised by childhood onset, visual failure, epilepti... Read More about Neurodegeneration and Epilepsy in a Zebrafish Model of CLN3 Disease (Batten Disease).

Neuroprotective coordination of cell mitophagy by the ATPase Inhibitory Factor 1 (2016)
Journal Article
Matic, I., Cocco, S., Ferraina, C., Martin-Jimenez, R., Florenzano, F., Crosby, J., Lupi, R., Amadoro, G., Russell, C., Pignataro, G., Annunziato, L., Abramov, A. Y., & Campanella, M. (2016). Neuroprotective coordination of cell mitophagy by the ATPase Inhibitory Factor 1. Pharmacological Research, 103(1), 56-68. https://doi.org/10.1016/j.phrs.2015.10.010

The mitochondrial ATPase Inhibitory Factor 1 (hereafter referred to as IF1) blocks the reversal of the F1Fo-ATPsynthase to prevent detrimental consumption of cellular ATP and associated demise. Herein, we infer further its molecular physiology by ass... Read More about Neuroprotective coordination of cell mitophagy by the ATPase Inhibitory Factor 1.

An Fgf8-Dependent Bistable Cell Migratory Event Establishes CNS Asymmetry
Journal Article
Regan, J. C., Concha, M. L., Roussigne, M., Russell, C., & Wilson, S. W. An Fgf8-Dependent Bistable Cell Migratory Event Establishes CNS Asymmetry. Neuron, 61(1), 27-34. https://doi.org/10.1016/j.neuron.2008.11.030

Neuroanatomical and functional asymmetries are universal features of the vertebrate CNS, but how asymmetry is generated is unknown. Here we show that zebrafish fgf8 mutants do not elaborate forebrain asymmetries, demonstrated by the failure of the pa... Read More about An Fgf8-Dependent Bistable Cell Migratory Event Establishes CNS Asymmetry.