Department

Institute

Research Theme Neurons, Circuits and Networks

Research Focus

Keywords

Axon Degeneration

Axon & Neuronal Degeneration

Axon Loss

Clinical Conditions

Motor neurone disease

Hereditary spastic paraplegia

Other

Equipment & Techniques

Cell culture

Enzyme assays

Fluorescence microscopy

Immunohistochemistry

Recombinant protein expression

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Dr Jonathan Gilley

University Position

Senior Research Associate

Interests

My principle research area is investigation of the molecular mechanisms involved in axon degeneration after injury and in disease.

Key Publications

Enrichment of SARM1 alleles encoding variants with constitutively hyperactive NADase in patients with ALS and other motor nerve disorders.

DOI: http://doi.org/10.7554/eLife.70905
Journal: Elife
E-pub date: 19 Nov 2021
Authors: J Gilley, O Jackson, M Pipis, MA Estiar, A Al-Chalabi, MC Danzi, KR van Eijk, SA Goutman, MB Harms, H Houlden, A Iacoangeli, J Kaye, L Lima, Queen Square Genomics, J Ravits, GA Rouleau, R Schüle, J Xu, S Züchner, J Cooper-Knock, Z Gan-Or, MM Reilly, MP Coleman

Homozygous NMNAT2 mutation in sisters with polyneuropathy and erythromelalgia.

DOI: http://doi.org/10.1016/j.expneurol.2019.112958
Journal: Exp Neurol
E-pub date: 1 Oct 2019
Authors: P Huppke, E Wegener, J Gilley, C Angeletti, I Kurth, JPH Drenth, C Stadelmann, A Barrantes-Freer, W Brück, H Thiele, P Nürnberg, J Gärtner, G Orsomando, MP Coleman

Severe biallelic loss-of-function mutations in nicotinamide mononucleotide adenylyltransferase 2 (NMNAT2) in two fetuses with fetal akinesia deformation sequence.

DOI: http://doi.org/10.1016/j.expneurol.2019.112961
Journal: Exp Neurol
E-pub date: 1 Oct 2019
Authors: M Lukacs, J Gilley, Y Zhu, G Orsomando, C Angeletti, J Liu, X Yang, J Park, RJ Hopkin, MP Coleman, RG Zhai, RW Stottmann

Endogenous Nmnat2 is an essential survival factor for maintenance of healthy axons.

DOI: http://doi.org/10.1371/journal.pbio.1000300
Journal: PLoS Biol
E-pub date: 26 Jan 2010
Authors: J Gilley, MP Coleman

Publications

Natural variants of human SARM1 cause both intrinsic and dominant loss-of-function influencing axon survival.

DOI: http://doi.org/10.1038/s41598-022-18052-8
Journal: Scientific Reports
E-pub date: 16 Aug 2022
Authors: M Ademi, X Yang, MP Coleman, J Gilley

SARM1 is a multi-functional NAD(P)ase with prominent base exchange activity, all regulated bymultiple physiologically relevant NAD metabolites.

DOI: http://doi.org/10.1016/j.isci.2022.103812
Journal: iScience
E-pub date: 18 Feb 2022
Authors: C Angeletti, A Amici, J Gilley, A Loreto, AG Trapanotto, C Antoniou, E Merlini, MP Coleman, G Orsomando

Sarm1 haploinsufficiency or low expression levels after antisense oligonucleotides delay programmed axon degeneration.

DOI: http://doi.org/10.1016/j.celrep.2021.110108
Journal: Cell Rep
E-pub date: 14 Dec 2021
Authors: SA Gould, J Gilley, K Ling, P Jafar-Nejad, F Rigo, M Coleman

Neurotoxin-mediated potent activation of the axon degeneration regulator SARM1.

DOI: http://doi.org/10.7554/eLife.72823
Journal: Elife
E-pub date: 6 Dec 2021
Authors: A Loreto, C Angeletti, W Gu, A Osborne, B Nieuwenhuis, J Gilley, E Merlini, P Arthur-Farraj, A Amici, Z Luo, L Hartley-Tassell, T Ve, LM Desrochers, Q Wang, B Kobe, G Orsomando, MP Coleman

NAD+ cleavage activity by animal and plant TIR domains in cell death pathways.

DOI: http://doi.org/10.1126/science.aax1911
Journal: Science
E-pub date: 23 Aug 2019
Authors: S Horsefield, H Burdett, X Zhang, MK Manik, Y Shi, J Chen, T Qi, J Gilley, J-S Lai, MX Rank, LW Casey, W Gu, DJ Ericsson, G Foley, RO Hughes, T Bosanac, M von Itzstein, JP Rathjen, JD Nanson, M Boden, IB Dry, SJ Williams, BJ Staskawicz, MP Coleman, T Ve, PN Dodds, B Kobe

Low levels of NMNAT2 compromise axon development and survival.

DOI: http://doi.org/10.1093/hmg/ddy356
Journal: Hum Mol Genet
E-pub date: 1 Feb 2019
Authors: J Gilley, PR Mayer, G Yu, MP Coleman

Sarm1 Deletion, but Not WldS, Confers Lifelong Rescue in a Mouse Model of Severe Axonopathy.

DOI: http://doi.org/10.1016/j.celrep.2017.09.027
Journal: Cell Rep
E-pub date: 3 Oct 2017
Authors: J Gilley, RR Ribchester, MP Coleman

NMN Deamidase Delays Wallerian Degeneration and Rescues Axonal Defects Caused by NMNAT2 Deficiency In Vivo.

DOI: http://doi.org/10.1016/j.cub.2017.01.070
Journal: Curr Biol
E-pub date: 20 Mar 2017
Authors: M Di Stefano, A Loreto, G Orsomando, V Mori, F Zamporlini, RP Hulse, J Webster, LF Donaldson, M Gering, N Raffaelli, MP Coleman, J Gilley, L Conforti

A rise in NAD precursor nicotinamide mononucleotide (NMN) after injury promotes axon degeneration.

DOI: http://doi.org/10.1038/cdd.2014.164
Journal: Cell Death Differ
E-pub date: 1 May 2015
Authors: M Di Stefano, I Nascimento-Ferreira, G Orsomando, V Mori, J Gilley, R Brown, L Janeckova, ME Vargas, LA Worrell, A Loreto, J Tickle, J Patrick, JRM Webster, M Marangoni, FM Carpi, S Pucciarelli, F Rossi, W Meng, A Sagasti, RR Ribchester, G Magni, MP Coleman, L Conforti