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Items: 43


Use of zebrafish models to investigate rare human disease.

Adamson KI, Sheridan E, Grierson AJ.

J Med Genet. 2018 Oct;55(10):641-649. doi: 10.1136/jmedgenet-2018-105358. Epub 2018 Jul 31.


C9orf72 plays a central role in Rab GTPase-dependent regulation of autophagy.

Webster CP, Smith EF, Grierson AJ, De Vos KJ.

Small GTPases. 2018 Sep 3;9(5):399-408. doi: 10.1080/21541248.2016.1240495. Epub 2016 Oct 21. Review.


The C9orf72 protein interacts with Rab1a and the ULK1 complex to regulate initiation of autophagy.

Webster CP, Smith EF, Bauer CS, Moller A, Hautbergue GM, Ferraiuolo L, Myszczynska MA, Higginbottom A, Walsh MJ, Whitworth AJ, Kaspar BK, Meyer K, Shaw PJ, Grierson AJ, De Vos KJ.

EMBO J. 2016 Aug 1;35(15):1656-76. doi: 10.15252/embj.201694401. Epub 2016 Jun 22.


AAV9-mediated central nervous system-targeted gene delivery via cisterna magna route in mice.

Lukashchuk V, Lewis KE, Coldicott I, Grierson AJ, Azzouz M.

Mol Ther Methods Clin Dev. 2016 Feb 17;3:15055. doi: 10.1038/mtm.2015.55. eCollection 2016.


Altered age-related changes in bioenergetic properties and mitochondrial morphology in fibroblasts from sporadic amyotrophic lateral sclerosis patients.

Allen SP, Duffy LM, Shaw PJ, Grierson AJ.

Neurobiol Aging. 2015 Oct;36(10):2893-903. doi: 10.1016/j.neurobiolaging.2015.07.013. Epub 2015 Jul 11.


Early detection of motor dysfunction in the SOD1G93A mouse model of Amyotrophic Lateral Sclerosis (ALS) using home cage running wheels.

Bennett EJ, Mead RJ, Azzouz M, Shaw PJ, Grierson AJ.

PLoS One. 2014 Sep 30;9(9):e107918. doi: 10.1371/journal.pone.0107918. eCollection 2014.


Superoxide dismutase 1 mutation in a cellular model of amyotrophic lateral sclerosis shifts energy generation from oxidative phosphorylation to glycolysis.

Allen SP, Rajan S, Duffy L, Mortiboys H, Higginbottom A, Grierson AJ, Shaw PJ.

Neurobiol Aging. 2014 Jun;35(6):1499-509. doi: 10.1016/j.neurobiolaging.2013.11.025. Epub 2013 Dec 3.


The effect of SOD1 mutation on cellular bioenergetic profile and viability in response to oxidative stress and influence of mutation-type.

Richardson K, Allen SP, Mortiboys H, Grierson AJ, Wharton SB, Ince PG, Shaw PJ, Heath PR.

PLoS One. 2013 Jun 28;8(6):e68256. doi: 10.1371/journal.pone.0068256. Print 2013.


Axonal Transport Defects in a Mitofusin 2 Loss of Function Model of Charcot-Marie-Tooth Disease in Zebrafish.

Chapman AL, Bennett EJ, Ramesh TM, De Vos KJ, Grierson AJ.

PLoS One. 2013 Jun 26;8(6):e67276. doi: 10.1371/journal.pone.0067276. Print 2013.


S[+] Apomorphine is a CNS penetrating activator of the Nrf2-ARE pathway with activity in mouse and patient fibroblast models of amyotrophic lateral sclerosis.

Mead RJ, Higginbottom A, Allen SP, Kirby J, Bennett E, Barber SC, Heath PR, Coluccia A, Patel N, Gardner I, Brancale A, Grierson AJ, Shaw PJ.

Free Radic Biol Med. 2013 Aug;61:438-52. doi: 10.1016/j.freeradbiomed.2013.04.018. Epub 2013 Apr 19.


Tardbpl splicing rescues motor neuron and axonal development in a mutant tardbp zebrafish.

Hewamadduma CA, Grierson AJ, Ma TP, Pan L, Moens CB, Ingham PW, Ramesh T, Shaw PJ.

Hum Mol Genet. 2013 Jun 15;22(12):2376-86. doi: 10.1093/hmg/ddt082. Epub 2013 Feb 19.


No evidence for cardiac dysfunction in Kif6 mutant mice.

Hameed A, Bennett E, Ciani B, Hoebers LP, Milner R, Lawrie A, Francis SE, Grierson AJ.

PLoS One. 2013;8(1):e54636. doi: 10.1371/journal.pone.0054636. Epub 2013 Jan 23.


Discovery of Western European R1b1a2 Y chromosome variants in 1000 genomes project data: an online community approach.

Rocca RA, Magoon G, Reynolds DF, Krahn T, Tilroe VO, Op den Velde Boots PM, Grierson AJ.

PLoS One. 2012;7(7):e41634. doi: 10.1371/journal.pone.0041634. Epub 2012 Jul 24.


Molecular pathways of motor neuron injury in amyotrophic lateral sclerosis.

Ferraiuolo L, Kirby J, Grierson AJ, Sendtner M, Shaw PJ.

Nat Rev Neurol. 2011 Nov;7(11):616-30. doi: 10.1038/nrneurol.2011.152. Review.


Review: The role of mitochondria in the pathogenesis of amyotrophic lateral sclerosis.

Duffy LM, Chapman AL, Shaw PJ, Grierson AJ.

Neuropathol Appl Neurobiol. 2011 Jun;37(4):336-52. doi: 10.1111/j.1365-2990.2011.01166.x. Review.


Impairment of the tRNA-splicing endonuclease subunit 54 (tsen54) gene causes neurological abnormalities and larval death in zebrafish models of pontocerebellar hypoplasia.

Kasher PR, Namavar Y, van Tijn P, Fluiter K, Sizarov A, Kamermans M, Grierson AJ, Zivkovic D, Baas F.

Hum Mol Genet. 2011 Apr 15;20(8):1574-84. doi: 10.1093/hmg/ddr034. Epub 2011 Jan 27.


Systemic delivery of scAAV9 expressing SMN prolongs survival in a model of spinal muscular atrophy.

Valori CF, Ning K, Wyles M, Mead RJ, Grierson AJ, Shaw PJ, Azzouz M.

Sci Transl Med. 2010 Jun 9;2(35):35ra42. doi: 10.1126/scitranslmed.3000830.


Mutations in CHMP2B in lower motor neuron predominant amyotrophic lateral sclerosis (ALS).

Cox LE, Ferraiuolo L, Goodall EF, Heath PR, Higginbottom A, Mortiboys H, Hollinger HC, Hartley JA, Brockington A, Burness CE, Morrison KE, Wharton SB, Grierson AJ, Ince PG, Kirby J, Shaw PJ.

PLoS One. 2010 Mar 24;5(3):e9872. doi: 10.1371/journal.pone.0009872.


Direct evidence for axonal transport defects in a novel mouse model of mutant spastin-induced hereditary spastic paraplegia (HSP) and human HSP patients.

Kasher PR, De Vos KJ, Wharton SB, Manser C, Bennett EJ, Bingley M, Wood JD, Milner R, McDermott CJ, Miller CC, Shaw PJ, Grierson AJ.

J Neurochem. 2009 Jul;110(1):34-44. doi: 10.1111/j.1471-4159.2009.06104.x. Epub 2009 Apr 22.


Role of axonal transport in neurodegenerative diseases.

De Vos KJ, Grierson AJ, Ackerley S, Miller CC.

Annu Rev Neurosci. 2008;31:151-73. doi: 10.1146/annurev.neuro.31.061307.090711. Review.


Familial amyotrophic lateral sclerosis-linked SOD1 mutants perturb fast axonal transport to reduce axonal mitochondria content.

De Vos KJ, Chapman AL, Tennant ME, Manser C, Tudor EL, Lau KF, Brownlees J, Ackerley S, Shaw PJ, McLoughlin DM, Shaw CE, Leigh PN, Miller CCJ, Grierson AJ.

Hum Mol Genet. 2007 Nov 15;16(22):2720-2728. doi: 10.1093/hmg/ddm226. Epub 2007 Aug 28.


Quantitative analysis of tau isoform transcripts in sporadic tauopathies.

Connell JW, Rodriguez-Martin T, Gibb GM, Kahn NM, Grierson AJ, Hanger DP, Revesz T, Lantos PL, Anderton BH, Gallo JM.

Brain Res Mol Brain Res. 2005 Jun 13;137(1-2):104-9. Epub 2005 Mar 29.


Mitochondrial function and actin regulate dynamin-related protein 1-dependent mitochondrial fission.

De Vos KJ, Allan VJ, Grierson AJ, Sheetz MP.

Curr Biol. 2005 Apr 12;15(7):678-83.


p38alpha stress-activated protein kinase phosphorylates neurofilaments and is associated with neurofilament pathology in amyotrophic lateral sclerosis.

Ackerley S, Grierson AJ, Banner S, Perkinton MS, Brownlees J, Byers HL, Ward M, Thornhill P, Hussain K, Waby JS, Anderton BH, Cooper JD, Dingwall C, Leigh PN, Shaw CE, Miller CC.

Mol Cell Neurosci. 2004 Jun;26(2):354-64.


Parkinson's disease alpha-synuclein mutations exhibit defective axonal transport in cultured neurons.

Saha AR, Hill J, Utton MA, Asuni AA, Ackerley S, Grierson AJ, Miller CC, Davies AM, Buchman VL, Anderton BH, Hanger DP.

J Cell Sci. 2004 Mar 1;117(Pt 7):1017-24.


Hereditary spastic paraparesis: disrupted intracellular transport associated with spastin mutation.

McDermott CJ, Grierson AJ, Wood JD, Bingley M, Wharton SB, Bushby KM, Shaw PJ.

Ann Neurol. 2003 Dec;54(6):748-59.


The cellular and molecular pathology of the motor system in hereditary spastic paraparesis due to mutation of the spastin gene.

Wharton SB, McDermott CJ, Grierson AJ, Wood JD, Gelsthorpe C, Ince PG, Shaw PJ.

J Neuropathol Exp Neurol. 2003 Nov;62(11):1166-77.


Neurofilament heavy chain side arm phosphorylation regulates axonal transport of neurofilaments.

Ackerley S, Thornhill P, Grierson AJ, Brownlees J, Anderton BH, Leigh PN, Shaw CE, Miller CC.

J Cell Biol. 2003 May 12;161(3):489-95.


Charcot-Marie-Tooth disease neurofilament mutations disrupt neurofilament assembly and axonal transport.

Brownlees J, Ackerley S, Grierson AJ, Jacobsen NJ, Shea K, Anderton BH, Leigh PN, Shaw CE, Miller CC.

Hum Mol Genet. 2002 Nov 1;11(23):2837-44.


Selective loss of neurofilament expression in Cu/Zn superoxide dismutase (SOD1) linked amyotrophic lateral sclerosis.

Menzies FM, Grierson AJ, Cookson MR, Heath PR, Tomkins J, Figlewicz DA, Ince PG, Shaw PJ.

J Neurochem. 2002 Sep;82(5):1118-28.


Axonal transport of neurofilaments in normal and disease states.

Miller CC, Ackerley S, Brownlees J, Grierson AJ, Jacobsen NJ, Thornhill P.

Cell Mol Life Sci. 2002 Feb;59(2):323-30. Review.


Phosphorylation of thr(668) in the cytoplasmic domain of the Alzheimer's disease amyloid precursor protein by stress-activated protein kinase 1b (Jun N-terminal kinase-3).

Standen CL, Brownlees J, Grierson AJ, Kesavapany S, Lau KF, McLoughlin DM, Miller CC.

J Neurochem. 2001 Jan;76(1):316-20.


Glutamate slows axonal transport of neurofilaments in transfected neurons.

Ackerley S, Grierson AJ, Brownlees J, Thornhill P, Anderton BH, Leigh PN, Shaw CE, Miller CC.

J Cell Biol. 2000 Jul 10;150(1):165-76.


Polyglutamine repeat length influences human androgen receptor/c-Jun mediated transcription.

Grierson AJ, Mootoosamy RC, Miller CC.

Neurosci Lett. 1999 Dec 17;277(1):9-12.


An integrated map of chromosome 18 CAG trinucleotide repeat loci.

Grierson AJ, van Groenigen M, Groot NP, Lindblad K, Hoovers JM, Schalling M, de Belleroche J, Baas F.

Eur J Hum Genet. 1999 Jan;7(1):12-9.


The gene for the human Src-like adaptor protein (hSLAP) is located within the 64-kb intron of the thyroglobulin gene.

Meijerink PH, Yanakiev P, Zorn I, Grierson AJ, Bikker H, Dye D, Kalaydjieva L, Baas F.

Eur J Biochem. 1998 Jun 1;254(2):297-303.


Cyclin D2 interacts with cdk-5 and modulates cellular cdk-5/p35 activity.

Guidato S, McLoughlin DM, Grierson AJ, Miller CC.

J Neurochem. 1998 Jan;70(1):335-40.


Investigation of the RB-1 tumour suppressor gene in a United Kingdom series of non-Hodgkin's lymphomas.

Grierson AJ, Hodgkins MA, Hancock BW, Goepel JR, Royds J, Goyns MH.

Leuk Lymphoma. 1996 Oct;23(3-4):353-63.


Gestational choriocarcinoma of the ovary diagnosed by analysis of tumour DNA.

Lorigan PC, Grierson AJ, Goepel JR, Coleman RE, Goyns MH.

Cancer Lett. 1996 Jun 24;104(1):27-30.


Investigation of the activation state of the X chromosome in non-Hodgkin's lymphomas.

Grierson AJ, Hammond DW, Goepel JR, Hancock BW, Goyns MH.

Ann Oncol. 1994;5 Suppl 1:47-50.


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