S
Steven Ackerley
Researcher at King's College London
Publications - 20
Citations - 5601
Steven Ackerley is an academic researcher from King's College London. The author has contributed to research in topics: Axoplasmic transport & Neurofilament. The author has an hindex of 18, co-authored 20 publications receiving 5143 citations. Previous affiliations of Steven Ackerley include Medical Research Council.
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Journal ArticleDOI
TDP-43 Mutations in Familial and Sporadic Amyotrophic Lateral Sclerosis
Jemeen Sreedharan,Ian P. Blair,Vineeta B. Tripathi,Xun Hu,Caroline Vance,Boris Rogelj,Steven Ackerley,Steven Ackerley,Jennifer C Durnall,Kelly L. Williams,Emanuele Buratti,Francisco E. Baralle,Jacqueline de Belleroche,J. Douglas Mitchell,P. Nigel Leigh,Ammar Al-Chalabi,Christopher C.J. Miller,Christopher C.J. Miller,Garth A. Nicholson,Garth A. Nicholson,Christopher Shaw +20 more
TL;DR: The evidence suggests a pathophysiological link between TDP-43 and ALS, and neighboring mutations in a highly conserved region of TARDBP in sporadic and familial ALS cases.
Journal ArticleDOI
Role of Axonal Transport in Neurodegenerative Diseases
TL;DR: The role of axonal transport in neurodegenerative disease is reviewed and disruption of axon transport is an early and perhaps causative event in many of these diseases.
Journal ArticleDOI
Familial amyotrophic lateral sclerosis-linked SOD1 mutants perturb fast axonal transport to reduce axonal mitochondria content
Kurt J. De Vos,Kurt J. De Vos,Anna L. Chapman,Maria E. Tennant,Catherine Manser,Elizabeth L. Tudor,Kwok-Fai Lau,Janet Brownlees,Steven Ackerley,Pamela J. Shaw,Declan M. McLoughlin,Christopher Shaw,P. Nigel Leigh,Christopher C.J. Miller,Andrew J. Grierson +14 more
TL;DR: Time-lapse microscopy is used to monitor for the first time the effect of mutant S OD1 on fast axonal transport (FAT) of bona fide cargoes in living neurons and finds that mutant SOD1 damages transport of both mitochondria and MBOs, and that the precise details of this damage are cargo-specific.
Journal ArticleDOI
VAPB interacts with the mitochondrial protein PTPIP51 to regulate calcium homeostasis
Kurt J. De Vos,Gábor M. Mórotz,Radu Stoica,Elizabeth L. Tudor,Kwok-Fai Lau,Steven Ackerley,Alice Warley,Christopher Shaw,Christopher C.J. Miller +8 more
TL;DR: It is shown that VAPB interacts with the outer mitochondrial membrane protein, protein tyrosine phosphatase-interacting protein 51 (PTPIP51), and VAPBP56S has altered binding to PTPIP 51 and increases Ca2+ uptake by mitochondria following release from ER stores.
Journal ArticleDOI
Charcot-Marie-Tooth disease neurofilament mutations disrupt neurofilament assembly and axonal transport
Janet Brownlees,Steven Ackerley,Andrew J. Grierson,Nick J.O. Jacobsen,Kerry Shea,Brian H. Anderton,P. Nigel Leigh,Christopher Shaw,Christopher C.J. Miller +8 more
TL;DR: It is demonstrated that aberrant neurofilaments assembly and transport can induce neurological disease, and further implicate defective neurofilament metabolism in the pathogenesis of human neurodegenerative diseases.