Publications
2023
Bennett CL, Dastidar S, Arnold FJ, McKinstry SU, Stockford C, Freibaum BD, Sopher BL, Wu M, Seidner G, Joiner W, Taylor JP, West RJH*, La Spada AR*. Senataxin helicase, the causal gene defect in ALS4, is a significant modifier of C9orf72 ALS G4C2 and arginine-containing dipeptide repeat toxicity. Acta Neuropathol Commun. 2023 Oct 17;11(1):164. doi: 10.1186/s40478-023-01665-z. PMID: 37845749; PMCID: PMC10580588. *Co-senior author
2022
Sharpe JL, Harper NS, West RJH. Identification and Monitoring of Nucleotide Repeat Expansions Using Southern Blotting in Drosophila Models of C9orf72 Motor Neuron Disease and Frontotemporal Dementia. Bio Protoc. 2022 May 20;12(10):e4424. doi: 10.21769/BioProtoc.4424. PMID: 35813024; PMCID: PMC9183971.
2021
Sharpe JL, Harper NS, Garner DR, West RJH. Modeling C9orf72-Related Frontotemporal Dementia and Amyotrophic Lateral Sclerosis in Drosophila. Front Cell Neurosci. 2021 Oct 21;15:770937. doi: 10.3389/fncel.2021.770937. PMID: 34744635; PMCID: PMC8566814.
2020
Ugbode C and West RJH. Lessons learned from CHMP2B, implications for frontotemporal dementia and amyotrophic lateral sclerosis. Neurobiol Dis. 2021 Jan;147:105144. doi: 10.1016/j.nbd.2020.105144. Epub 2020 Nov 2. PMID: 33144171.
 West RJH*, Sharpe JL, Voelzmann A, Munro AL, Hahn I, Baines RA, Pickering-Brown S*. Co-expression of C9orf72 related dipeptide-repeats over 1000 repeat units reveals age- and combination-specific phenotypic profiles in Drosophila. Acta Neuropathol Commun. 2020 Sep 7;8(1):158. doi: 10.1186/s40478-020-01028-y. PMID: 32894207; PMCID: PMC7487709. * co-corresponding author
Lu Y, West RJH, Pons M, Sweeney ST, Gao FB. Ik2/TBK1 and Hook/Dynein, an adaptor complex for early endosome transport, are genetic modifiers of FTD-associated mutant CHMP2B toxicity in Drosophila. Sci Rep. 2020 Aug 26;10(1):14221. doi: 10.1038/s41598-020-71097-5. PMID: 32848189; PMCID: PMC7450086.
West RJH, Ugbode C, Fort-Aznar L, Sweeney ST. Neuroprotective activity of ursodeoxycholic acid in CHMP2BIntron5 models of frontotemporal dementia. Neurobiol Dis. 2020 Oct;144:105047. doi: 10.1016/j.nbd.2020.105047. Epub 2020 Aug 13. PMID: 32801000; PMCID: PMC7491204.
2018
Oswald MC, Brooks PS, Zwart MF, Mukherjee A, West RJH, Giachello CN, Morarach K, Baines RA, Sweeney ST, Landgraf M. Reactive oxygen species regulate activity-dependent neuronal plasticity in Drosophila. Elife. 2018 Dec 17;7. pii: e39393. doi: 10.7554/eLife.39393.
Vilidaite G, Norcia AM, West RJH, Elliott CJH, Wade AR, Baker DH. Autism sensory dysfunction in an evolutionarily conserved system. Proc Biol Sci. 2018 Dec 19;285(1893):20182255. doi: 10.1098/rspb.2018.2255.
West RJH, Briggs L, Perona Fjeldstad M, Ribchester RR, Sweeney ST. Sphingolipids regulate neuromuscular synapse structure and function in Drosophila. J Comp Neurol. 2018 Sep 1;526(13):1995-2009. doi: 10.1002/cne.24466. Epub 2018 Aug 2. PubMed PMID: 29761896; PubMed Central PMCID: PMC6175220.
West RJH, Ugbode C, Gao FB, Sweeney ST. The pro-apoptotic JNK scaffold POSH/SH3RF1 mediates CHMP2BIntron5-associated toxicity in animal models of frontotemporal dementia. Hum Mol Genet. 2018 Apr 15;27(8):1382-1395. doi: 10.1093/hmg/ddy048. PubMed PMID: 29432529.
Himmelberg MM, West RJH, Elliott CJH, Wade AR. Abnormal visual gain control and excitotoxicity in early-onset Parkinson's disease Drosophila models. J Neurophysiol. 2018 Mar 1;119(3):957-970. doi: 10.1152/jn.00681.2017. Epub 2017 Nov 15. PubMed PMID: 29142100; PubMed Central PMCID: PMC5899316.
Himmelberg MM, West RJH, Wade AR, Elliott CJH. A perceptive plus in Parkinson's disease. Mov Disord. 2018 Feb;33(2):248. doi: 10.1002/mds.27240. Epub 2018 Jan 14. PubMed PMID: 29333617; PubMed Central PMCID: PMC5838541.
2015
Oswald MC, West RJH, Lloyd-Evans E, Sweeney ST. Identification of dietary alanine toxicity and trafficking dysfunction in a Drosophila model of hereditary sensory and autonomic neuropathy type 1. Hum Mol Genet. 2015 Dec 15;24(24):6899-909. doi: 10.1093/hmg/ddv390. Epub 2015 Sep 22. PubMed PMID: 26395456; PubMed Central PMCID: PMC4654049.
West RJH, Elliott CJ, Wade AR. Classification of Parkinson's Disease Genotypes in Drosophila Using Spatiotemporal Profiling of Vision. Sci Rep. 2015 Nov 24;5:16933. doi: 10.1038/srep16933. PubMed PMID: 26597171; PubMed Central PMCID: PMC4657034.
West RJH, Eyeing up Drosophila models of frontotemporal dementia: Identifying conserved mechanisms in disease pathology. Future Neurology. 2015 Nov 10(6):507-510. 10.2217/fnl.15.30
Katzemich A, West RJH, Fukuzawa A, Sweeney ST, Gautel M, Sparrow J, Bullard B. Binding partners of the kinase domains in Drosophila obscurin and their effect on the structure of the flight muscle. J Cell Sci. 2015 Sep 15;128(18):3386-97. doi: 10.1242/jcs.170639. Epub 2015 Aug 6. PubMed PMID: 26251439; PubMed Central PMCID: PMC4582401.
West RJH, Furmston R, Williams CA, Elliott CJ. Neurophysiology of Drosophila models of Parkinson's disease. Parkinsons Dis. 2015;2015:381281. doi: 10.1155/2015/381281. Epub 2015 Apr 20. Review. PubMed PMID: 25960916; PubMed Central PMCID: PMC4414211.
West RJH, Lu Y, Marie B, Gao FB, Sweeney ST. Rab8, POSH, and TAK1 regulate synaptic growth in a Drosophila model of frontotemporal dementia. J Cell Biol. 2015 Mar 30;208(7):931-47. doi: 10.1083/jcb.201404066. Epub 2015 Mar 23. PubMed PMID: 25800055; PubMed Central PMCID: PMC4384727.