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Research Outputs 1993 2019

2019

Is cytoplasmic FUS a feature of all ALS?

Vidal, M. & Atkin, J. D., 1 Sep 2019, In : Brain : a journal of neurology. 142, 9, p. 2546-2549 4 p.

Research output: Contribution to journalArticleResearchpeer-review

Amyotrophic Lateral Sclerosis
Brain

Motor neuron abnormalities correlate with impaired movement in zebrafish that express mutant superoxide dismutase 1

Robinson, K. J., Yuan, K. C., Don, E. K., Hogan, A. L., Winnick, C. G., Tym, M. C., Lucas, C. W., Shahheydari, H., Watchon, M., Blair, I. P., Atkin, J. D., Nicholson, G. A., Cole, N. J. & Laird, A. S., 1 Feb 2019, In : Zebrafish. 16, 1, p. 8-14 7 p.

Research output: Contribution to journalArticleResearchpeer-review

Open Access
File
Motor Neurons
Zebrafish
motor neurons
Danio rerio
superoxide dismutase

Motor neuron susceptibility in ALS/FTD

Ragagnin, A. M. G., Shadfar, S., Vidal, M., Jamali, M. S. & Atkin, J. D., 27 Jun 2019, In : Frontiers in Neuroscience. 13, JUN, p. 1-37 37 p., 532.

Research output: Contribution to journalReview articleResearchpeer-review

Open Access
File
Amyotrophic Lateral Sclerosis
Motor Neurons
Intrinsic Factor
Neurodegenerative Diseases
Brain Stem

Protein disulphide isomerase is associated with mutant SOD1 in canine degenerative myelopathy

Chang, R. C., Parakh, S., Coates, J. R., Long, S. & Atkin, J. D., 2 Jan 2019, In : NeuroReport. 30, 1, p. 8-13 6 p.

Research output: Contribution to journalArticleResearchpeer-review

Protein Disulfide-Isomerases
Spinal Cord Diseases
Canidae
Amyotrophic Lateral Sclerosis
Endoplasmic Reticulum Stress
2018

Dysfunction of optineurin in amyotrophic lateral sclerosis and glaucoma

Toth, R. P. & Atkin, J. D., 23 May 2018, In : Frontiers in Immunology. 9, p. 1-19 19 p., 1017.

Research output: Contribution to journalReview articleResearchpeer-review

Open Access
File
Amyotrophic Lateral Sclerosis
Glaucoma
Autophagy
Neurodegenerative Diseases
Mutation

ERp57 is protective against mutant SOD1-induced cellular pathology in amyotrophic lateral sclerosis

Parakh, S., Jagaraj, C. J., Vidal, M., Ragagnin, A. M. G., Perri, E. R., Konopka, A., Toth, R. P., Galper, J., Blair, I. P., Thomas, C. J., Walker, A. K., Yang, S., Spencer, D. M. & Atkin, J. D., 15 Apr 2018, In : Human molecular genetics. 27, 8, p. 1311-1331 21 p.

Research output: Contribution to journalArticleResearchpeer-review

Amyotrophic Lateral Sclerosis
Endoplasmic Reticulum Stress
Pathology
Apoptosis
DNA-Binding Proteins

Pathogenic mutation in the ALS/FTD gene, CCNF, causes elevated Lys48-linked ubiquitylation and defective autophagy

Lee, A., Rayner, S. L., Gwee, S. S. L., De Luca, A., Shahheydari, H., Sundaramoorthy, V., Ragagnin, A., Morsch, M., Radford, R., Galper, J., Freckleton, S., Shi, B., Walker, A. K., Don, E. K., Cole, N. J., Yang, S., Williams, K. L., Yerbury, J. J., Blair, I. P., Atkin, J. D. & 2 othersMolloy, M. P. & Chung, R. S., Jan 2018, In : Cellular and Molecular Life Sciences. 75, 2, p. 335–354 20 p.

Research output: Contribution to journalArticleResearchpeer-review

Cyclins
Ubiquitination
Autophagy
Mutation
Genes

Rab-dependent cellular trafficking and amyotrophic lateral sclerosis

Parakh, S., Perri, E. R., Jagaraj, C. J., Ragagnin, A. M. G. & Atkin, J. D., 2 Nov 2018, In : Critical Reviews in Biochemistry and Molecular Biology. 53, 6, p. 623-651 29 p.

Research output: Contribution to journalReview articleResearchpeer-review

rab GTP-Binding Proteins
Amyotrophic Lateral Sclerosis
Neurons
Defects
Intracellular Membranes

SOD1 mutations causing familial amyotrophic lateral sclerosis induce toxicity in astrocytes: evidence for bystander effects in a continuum of astrogliosis

Wallis, N., Lau, C. L., Farg, M. A., Atkin, J. D., Beart, P. M. & O’Shea, R. D., 1 Jan 2018, In : Neurochemical Research. 43, 1, p. 166-179 14 p.

Research output: Contribution to journalArticleResearchpeer-review

Bystander Effect
Astrocytes
Toxicity
Mutation
Amyotrophic Lateral Sclerosis

The emerging role of DNA damage in the pathogenesis of the C9Orf72 repeat expansion in amyotrophic lateral sclerosis

Konopka, A. & Atkin, J. D., 12 Oct 2018, In : International Journal of Molecular Sciences. 19, 10, p. 1-21 21 p., 3137.

Research output: Contribution to journalReview articleResearchpeer-review

Open Access
File
pathogenesis
Chromosomes, Human, Pair 9
chromosomes
Amyotrophic Lateral Sclerosis
Chromosomes
2017

A novel amyotrophic lateral sclerosis mutation in OPTN induces ER stress and Golgi fragmentation in vitro

Fifita, J. A., Williams, K. L., Sundaramoorthy, V., Mccann, E. P., Nicholson, G. A., Atkin, J. D. & Blair, I. P., 2 Jan 2017, In : Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration. 18, 1-2, p. 126-133 8 p.

Research output: Contribution to journalArticleResearchpeer-review

Amyotrophic Lateral Sclerosis
Mutation
Genes
In Vitro Techniques

Casein kinase II phosphorylation of cyclin F at serine 621 regulates the Lys48-ubiquitylation E3 ligase activity of the SCF(cyclin F) complex

Lee, A., Rayner, S. L., De Luca, A., Gwee, S. S. L., Morsch, M., Sundaramoorthy, V., Shahheydari, H., Ragagnin, A., Shi, B., Yang, S., Williams, K. L., Don, E. K., Walker, A. K., Zhang, K. Y., Yerbury, J. J., Cole, N. J., Atkin, J. D., Blair, I. P., Molloy, M. P. & Chung, R. S., 2017, In : Open Biology. 7, 10, p. 1-11 11 p., 170058.

Research output: Contribution to journalArticleResearchpeer-review

Open Access
File
Casein Kinase II
Phosphorylation
Cyclins
Ubiquitin-Protein Ligases
Ubiquitination

Protein Disulphide Isomerases: emerging roles of PDI and ERp57 in the nervous system and as therapeutic targets for ALS

Perri, E., Parakh, S. & Atkin, J., 2 Jan 2017, In : Expert Opinion on Therapeutic Targets. 21, 1, p. 37-49 13 p.

Research output: Contribution to journalReview articleResearchpeer-review

Protein Disulfide-Isomerases
Amyotrophic Lateral Sclerosis
Neurology
Endoplasmic Reticulum
Nervous System

Protein quality control and the amyotrophic lateral sclerosis/frontotemporal dementia continuum

Shahheydari, H., Ragagnin, A., Walker, A. K., Toth, R. P., Vidal, M., Jagaraj, C. J., Perri, E. R., Konopka, A., Sultana, J. M. & Atkin, J. D., 10 May 2017, In : Frontiers in Molecular Neuroscience. 10, p. 1-25 25 p., 119.

Research output: Contribution to journalArticleResearchpeer-review

Open Access
File
Quality Control
Proteins
Neurodegenerative Diseases
Endoplasmic Reticulum-Associated Degradation
Molecular Chaperones

The DNA damage response (DDR) is induced by the C9orf72 repeat expansion in amyotrophic lateral sclerosis

Farg, M. A., Konopka, A., Soo, K. Y., Ito, D. & Atkin, J. D., 1 Aug 2017, In : Human molecular genetics. 26, 15, p. 2882-2896 15 p.

Research output: Contribution to journalArticleResearchpeer-review

Amyotrophic Lateral Sclerosis
DNA Damage
Ataxia Telangiectasia
Dipeptides
Motor Neurons
2016

CCNF mutations in amyotrophic lateral sclerosis and frontotemporal dementia

Williams, K. L., Topp, S., Yang, S., Smith, B., Fifita, J. A., Warraich, S. T., Zhang, K. Y., Farrawell, N., Vance, C., Hu, X., Chesi, A., Leblond, C. S., Lee, A., Rayner, S. L., Sundaramoorthy, V., Dobson-Stone, C., Molloy, M. P., Van Blitterswijk, M., Dickson, D. W., Petersen, R. C. & 48 othersGraff-Radford, N. R., Boeve, B. F., Murray, M. E., Pottier, C., Don, E., Winnick, C., McCann, E. P., Hogan, A., Daoud, H., Levert, A., Dion, P. A., Mitsui, J., Ishiura, H., Takahashi, Y., Goto, J., Kost, J., Gellera, C., Gkazi, A. S., Miller, J., Stockton, J., Brooks, W. S., Boundy, K., Polak, M., Muñoz-Blanco, J. L., Esteban-Pérez, J., Rábano, A., Hardiman, O., Morrison, K. E., Ticozzi, N., Silani, V., De Belleroche, J., Glass, J. D., Kwok, J. B. J., Guillemin, G. J., Chung, R. S., Tsuji, S., Brown, R. H., García-Redondo, A., Rademakers, R., Landers, J. E., Gitler, A. D., Rouleau, G. A., Cole, N. J., Yerbury, J. J., Atkin, J. D., Shaw, C. E., Nicholson, G. A. & Blair, I. P., 15 Apr 2016, In : Nature Communications. 7, p. 1-8 8 p., 11253.

Research output: Contribution to journalArticleResearchpeer-review

Open Access
File
Ubiquitinated Proteins
mutations
Ubiquitin-Protein Ligase Complexes
proteins
Mutation

Efficacy of peptide nucleic acid and selected conjugates against specific cellular pathologies of amyotrophic lateral sclerosis

Browne, E. C., Parakh, S., Duncan, L. F., Langford, S. J., Atkin, J. D. & Abbott, B. M., 1 Apr 2016, In : Bioorganic and Medicinal Chemistry. 24, 7, p. 1520-1527 8 p.

Research output: Contribution to journalArticleResearchpeer-review

Peptide Nucleic Acids
Amyotrophic Lateral Sclerosis
Pathology
Superoxide Dismutase
Nucleic acid sequences

Guidelines for the use and interpretation of assays for monitoring autophagy (3rd edition)

Klionsky, D. J., Abdelmohsen, K., Abe, A., Abedin, M. J., Abeliovich, H., Arozena, A. A., Adachi, H., Adams, C. M., Adams, P. D., Adeli, K., Adhihetty, P. J., Adler, S. G., Agam, G., Agarwal, R., Aghi, M. K., Agnello, M., Agostinis, P., Aguilar, P. V., Aguirre-Ghiso, J., Airoldi, E. M. & 224 othersAit-Si-Ali, S., Akematsu, T., Akporiaye, E. T., Al-Rubeai, M., Albaiceta, G. M., Albanese, C., Albani, D., Albert, M. L., Aldudo, J., Algül, H., Alirezaei, M., Alloza, I., Almasan, A., Almonte-Beceril, M., Alnemri, E. S., Alonso, C., Altan-Bonnet, N., Altieri, D. C., Alvarez, S., Alvarez-Erviti, L., Alves, S., Amadoro, G., Amano, A., Amantini, C., Ambrosio, S., Amelio, I., Amer, A. O., Amessou, M., Amon, A., An, Z., Anania, F. A., Andersen, S. U., Andley, U. P., Andreadi, C. K., Andrieu-Abadie, N., Anel, A., Ann, D. K., Anoopkumar-Dukie, S., Antonioli, M., Aoki, H., Apostolova, N., Aquila, S., Aquilano, K., Araki, K., Arama, E., Aranda, A., Araya, J., Arcaro, A., Arias, E., Arimoto, H., Ariosa, A. R., Armstrong, J. L., Arnould, T., Arsov, I., Asanuma, K., Askanas, V., Asselin, E., Atarashi, R., Atherton, S. S., Atkin, J. D., Attardi, L. D., Auberger, P., Auburger, G., Aurelian, L., Autelli, R., Avagliano, L., Avantaggiati, M. L., Avrahami, L., Azad, N., Awale, S., Bachetti, T., Backer, J. M., Bae, D. H., Bae, J. S., Bae, O. N., Bae, S. H., Baehrecke, E. H., Baek, S. H., Baghdiguian, S., Bagniewska-Zadworna, A., Bai, H., Bai, J., Bai, X. Y., Bailly, Y., Balaji, K. N., Balduini, W., Ballabio, A., Balzan, R., Banerjee, R., Bánhegyi, G., Bao, H., Barbeau, B., Barrachina, M. D., Barreiro, E., Bartel, B., Bartolomé, A., Bassham, D. C., Bassi, M. T., Bast, R. C., Basu, A., Batista, M. T., Batoko, H., Battino, M., Bauckman, K., Baumgarner, B. L., Bayer, K. U., Beale, R., Beaulieu, J. F., Beck, G. R., Becker, C., Beckham, J. D., Bédard, P. A., Burke, R. E., Campbell, M., Chen, M., Chen, Q., Chen, W., Chen, W-J., Chen, W. Q., Chen, Y., Chen, Z., Chen, Z., Clarke, P. G. H., Dickson, R. C., Dong, Z., Du, L-L., Evans, T. J., Fan, J., Fisher, P. B., Gao, F., Han, F., Harris, J., He, R. R., Hoppe, T., Huang, K. H., Huang, W. P., Huang, Y-R., Jackson, W. T., Johnson, D. E., Jones, C., Karim, M. R., Kim, E. K., Kim, J. S., Kim, J. C., Kim, Y. S., Lee, J. K., Lee, J. H., Lee, M., Lee, S-J., Lee, S. Y., Lee, S. J., Lee, Y. H., Li, C., Li, F. J., Li, J., Li, M., Li, Q., Li, W., Lin, W. W., Liu, F., Liu, J. J., Liu, R. Y., Liu, W., Mihailidou, A. S., Mitchell, C. H., Moore, M. N., Morgan, M. J., Murray, J. T., Rimbach, G., Song, W., Sue, C. M., Sun, Y., Sun, Y., Sundaramoorthy, V., Sweeney, S. T., Taylor, G. S., Taylor, G. A., Taylor, M. J., Wang, B., Wang, J., Wang, M., Wang, M-Q., Wang, P. Y., Wang, S., Wang, T. F., Wang, X., Wang, X-W., Wang, X., Wang, Y., Wang, Y., Wang, Y-J., Wang, Y., Wang, Y. T., Wang, Y., White, A. R., Williams, K., Williamson, P. R., Wolvetang, E. J., Wong, R. W., Wong, V. K. W., Woodcock, E. A., Xu, F., Xu, J., Yang, J. M., Yang, Q., Yang, W. Y., Yang, Y., Yang, Y., Yu, X-F., Zhang, F., Zhang, H., Zhang, J., Zhang, J. P., Zhang, L., Zhang, L., Zhang, L., Zhang, M. Y., Zhang, Y., Zhao, M., Zhao, W-L., Zhao, Y. G., Zhao, Z., Zheng, X-L. & Zhu, X-F., 21 Jan 2016, In : Autophagy. 12, 1, p. 1-222 222 p.

Research output: Contribution to journalReview articleResearchpeer-review

NFκB is a central regulator of protein quality control in response to protein aggregation stresses via autophagy modulation

Nivon, M., Fort, L., Muller, P., Richet, E., Simon, S., Guey, B., Fournier, M., Arrigo, A. P., Hetz, C., Atkin, J. D. & Kretz-Remy, C., 1 Jun 2016, In : Molecular Biology of the Cell. 27, 11, p. 1712-1727 16 p.

Research output: Contribution to journalArticleResearchpeer-review

Open Access
File
Autophagy
Heat-Shock Proteins
Quality Control
Proteins
Poisons

Protein folding alterations in amyotrophic lateral sclerosis

Parakh, S. & Atkin, J. D., 1 Oct 2016, In : Brain Research. 1648, p. 633-649 17 p.

Research output: Contribution to journalReview articleResearchpeer-review

Protein Folding
Amyotrophic Lateral Sclerosis
Proteins
Neurodegenerative Diseases
Protein Disulfide-Isomerases

The C9ORF72 repeat expansion induces apoptosis by the DNA damage response (DDR)

Atkin, J., Aug 2016, In : Journal of Neurochemistry. 138, S1, p. 410 1 p.

Research output: Contribution to journalMeeting abstractResearch

The role of FTD/ALS associated protein, TDP-43, in neurite and synapse health and function

Atkinson, R., Leung, J., Fernandez-Martos, C., Atkin, J., Vickers, J. C. & King, A., Aug 2016, In : Journal of Neurochemistry. 138, S1, p. 418 1 p.

Research output: Contribution to journalMeeting abstractResearch

2015

Autophagy dysregulation by mutant fused in sarcoma-implications for amyotrophic lateral sclerosis

Soo, K. Y. & Atkin, J. D., 1 Oct 2015, In : Cell Death and Disease. 6, 10, p. 1-3 3 p., e1945.

Research output: Contribution to journalComment/opinionResearchpeer-review

Open Access
File

C9ORF72 expression and cellular localization over mouse development

Atkinson, R. A. K., Fernandez-Martos, C. M., Atkin, J. D., Vickers, J. C. & King, A. E., 25 Sep 2015, In : Acta neuropathologica communications. 3, p. 1-11 11 p., 59.

Research output: Contribution to journalArticleResearchpeer-review

Open Access
File
Protein Isoforms
Fluorescent Antibody Technique
Neurons
Growth Cones
Proteins

Defects in optineurin- and myosin VI-mediated cellular trafficking in amyotrophic lateral sclerosis

Sundaramoorthy, V., Walker, A. K., Tan, V., Fifita, J. A., McCann, E. P., Williams, K. L., Blair, I. P., Guillemin, G. J., Farg, M. A. & Atkin, J. D., 1 Jul 2015, In : Human molecular genetics. 24, 13, p. 3830-3846 17 p., ddv126.

Research output: Contribution to journalArticleResearchpeer-review

Amyotrophic Lateral Sclerosis
Motor Neurons
Lysosomes
Autophagy
Mutation

Golgi fragmentation in amyotrophic lateral sclerosis, an overview of possible triggers and consequences

Sundaramoorthy, V., Sultana, J. M. & Atkin, J. D., 2015, In : Frontiers in Neuroscience. 9, p. 1-11 11 p., 400.

Research output: Contribution to journalShort surveyResearchpeer-review

Open Access
File
Amyotrophic Lateral Sclerosis
Autophagy
Golgi Apparatus
Motor Neurons
Animal Disease Models

Novel roles for protein disulphide isomerase in disease states: a double edged sword?

Parakh, S. & Atkin, J. D., 2015, In : Frontiers in cell and developmental biology. 3, p. 1-11 11 p., 30.

Research output: Contribution to journalArticleResearchpeer-review

Open Access
File
Protein Disulfide-Isomerases
Post Translational Protein Processing
Endoplasmic Reticulum
Oxidation-Reduction

Phosphorylation of hnRNP K by cyclin-dependent kinase 2 controls cytosolic accumulation of TDP-43

Moujalled, D., James, J. L., Yang, S., Zhang, K., Duncan, C., Moujalled, D. M., Parker, S. J., Caragounis, A., Lidgerwood, G., Turner, B. J., Atkin, J. D., Grubman, A., Liddell, J. R., Proepper, C., Boeckers, T. M., Kanninen, K. M., Blair, I., Crouch, P. J. & White, A. R., 2015, In : Human molecular genetics. 24, 6, p. 1655-1669 15 p., ddu578.

Research output: Contribution to journalArticleResearchpeer-review

Cyclin-Dependent Kinase 2
Ribonucleoproteins
DNA-Binding Proteins
Phosphorylation
Amyotrophic Lateral Sclerosis

Rab1-dependent ER-Golgi transport dysfunction is a common pathogenic mechanism in SOD1, TDP-43 and FUS-associated ALS

Soo, K. Y., Halloran, M., Sundaramoorthy, V., Parakh, S., Toth, R. P., Southam, K. A., McLean, C. A., Lock, P., King, A., Farg, M. A. & Atkin, J. D., 1 Nov 2015, In : Acta Neuropathologica. 130, 5, p. 679-697 19 p.

Research output: Contribution to journalArticleResearchpeer-review

Amyotrophic Lateral Sclerosis
Endoplasmic Reticulum
Protein Transport
Motor Neurons
Endoplasmic Reticulum Stress

The unfolded protein response and the role of Protein Disulfide Isomerase in neurodegeneration

Perri, E. R., Thomas, C. J., Parakh, S., Spencer, D. M. & Atkin, J. D., 2015, In : Frontiers in cell and developmental biology. 3, p. 1-17 17 p., 80.

Research output: Contribution to journalArticleResearchpeer-review

Open Access
File
Protein Disulfide-Isomerases
Unfolded Protein Response
Endoplasmic Reticulum Stress
Endoplasmic Reticulum
Neurodegenerative Diseases
2014

C9ORF72, implicated in amytrophic lateral sclerosis and frontotemporal dementia, regulates endosomal trafficking

Farg, M. A., Sundaramoorthy, V., Sultana, J. M., Yang, S., Atkinson, R. A. K., Levina, V., Halloran, M. A., Gleeson, P. A., Blair, I. P., Soo, K. Y., King, A. E. & Atkin, J. D., 2014, In : Human molecular genetics. 23, 13, p. 3579-3595 17 p., ddu068.

Research output: Contribution to journalArticleResearchpeer-review

Frontotemporal Dementia
Motor Neuron Disease
Reading
Autophagy
Motor Neurons

Mutant SOD1 inhibits ER-Golgi transport in amyotrophic lateral sclerosis

Atkin, J. D., Farg, M. A., Soo, K. Y., Walker, A. K., Halloran, M., Turner, B. J., Nagley, P. & Horne, M. K., 2014, In : Journal of Neurochemistry. 129, 1, p. 190-204 15 p.

Research output: Contribution to journalArticleResearchpeer-review

Amyotrophic Lateral Sclerosis
Endoplasmic Reticulum
Endoplasmic Reticulum Stress
Apoptosis
Capsid Proteins
2013

ALS-associated TDP-43 induces endoplasmic reticulum stress, which drives cytoplasmic TDP-43 accumulation and stress granule formation

Walker, A. K., Soo, K. Y., Sundaramoorthy, V., Parakh, S., Ma, Y., Farg, M. A., Wallace, R. H., Crouch, P. J., Turner, B. J., Horne, M. K. & Atkin, J. D., 29 Nov 2013, In : PLoS ONE. 8, 11, p. 1-12 12 p., e81170.

Research output: Contribution to journalArticleResearchpeer-review

Open Access
File
Endoplasmic Reticulum Stress
DNA-binding proteins
Amyotrophic Lateral Sclerosis
DNA-Binding Proteins
endoplasmic reticulum

Ataxin-2 interacts with FUS and intermediate-length polyglutamine expansions enhance FUS-related pathology in amyotrophic lateral sclerosis

Farg, M. A., Soo, K. Y., Warraich, S. T., Sundaramoorthy, V., Blair, I. P. & Atkin, J. D., Feb 2013, In : Human molecular genetics. 22, 4, p. 717-728 12 p., dds479.

Research output: Contribution to journalArticleResearchpeer-review

Amyotrophic Lateral Sclerosis
Sarcoma
Pathology
Endoplasmic Reticulum Stress
Motor Neurons

Dysregulation of the complement cascade in the hSOD1G93A transgenic mouse model of amyotrophic lateral sclerosis

Lee, J. D., Kamaruzaman, N. A., Fung, J. N. T., Taylor, S. M., Turner, B. J., Atkin, J. D., Woodruff, T. M. & Noakes, P. G., 26 Sep 2013, In : Journal of Neuroinflammation. 10, p. 1-14 14 p., 119.

Research output: Contribution to journalArticleResearchpeer-review

Open Access
File
Amyotrophic Lateral Sclerosis
Transgenic Mice
Spinal Cord
Motor Neurons
Disease Progression

Extracellular wildtype and mutant SOD1 induces ER-Golgi pathology characteristic of amyotrophic lateral sclerosis in neuronal cells

Sundaramoorthy, V., Walker, A. K., Yerbury, J., Soo, K. Y., Farg, M. A., Hoang, V., Zeineddine, R., Spencer, D. & Atkin, J. D., Nov 2013, In : Cellular and Molecular Life Sciences. 70, 21, p. 4181-4195 15 p.

Research output: Contribution to journalArticleResearchpeer-review

Amyotrophic Lateral Sclerosis
Pathology
Motor Neurons
Cell Death
Golgi Apparatus

N-linked glycosylation modulates dimerization of protein disulfide isomerase family A member 2 (PDIA2)

Walker, A. K., Soo, K. Y., Levina, V., Talbo, G. H. & Atkin, J. D., Jan 2013, In : FEBS Journal. 280, 1, p. 233-243 11 p.

Research output: Contribution to journalArticleResearchpeer-review

Protein Disulfide-Isomerases
Glycosylation
Dimerization
Endoplasmic Reticulum
Post Translational Protein Processing

Redox regulation in amyotrophic lateral sclerosis

Parakh, S., Spencer, D. M., Halloran, M. A., Soo, K. Y. & Atkin, J. D., 2013, In : Oxidative Medicine and Cellular Longevity. 2013, p. 1-12 12 p., 408681.

Research output: Contribution to journalReview articleResearchpeer-review

Open Access
File
Amyotrophic Lateral Sclerosis
Oxidation-Reduction
Protein Disulfide-Isomerases
Motor Neurons
Oxidation

The role of S-nitrosylation and S-glutathionylation of protein disulphide isomerase in protein misfolding and neurodegeneration

Halloran, M., Parakh, S. & Atkin, J. D., 2013, In : International Journal of Cell Biology. 2013, p. 1-15 15 p., 797914.

Research output: Contribution to journalReview articleResearchpeer-review

Open Access
File
Protein Disulfide-Isomerases
Neurodegenerative Diseases
Proteins
Endoplasmic Reticulum Stress
Molecular Chaperones
2012

Bim links ER stress and apoptosis in cells expressing mutant SOD1 associated with amyotrophic lateral sclerosis

Soo, K. Y., Atkin, J. D., Farg, M., Walker, A. K., Horne, M. K. & Nagley, P., 16 Apr 2012, In : PLoS ONE. 7, 4, p. 1-11 11 p., e35413.

Research output: Contribution to journalArticleResearchpeer-review

Open Access
File
Endoplasmic Reticulum Stress
Amyotrophic Lateral Sclerosis
Bearings (structural)
endoplasmic reticulum
Superoxide Dismutase

Membrane trafficking defects as determinants of motor neuron susceptibility and degeneration in ALS

Turner, B. J. & Atkin, J. D., 2012, Motor Neuron Diseases: Causes, Classification and Treatments. Turner, B. J. & Atkin, J. B. (eds.). New York: Nova Science Publishers, p. 39-74 36 p.

Research output: Chapter in Book/Report/Conference proceedingChapterResearchpeer-review

Nerve Degeneration
Amyotrophic Lateral Sclerosis
Motor Neurons
Membranes
Molecular Motor Proteins

Motor neuron diseases: causes, classification, and treatments

Turner, B. J. (ed.) & Atkin, J. B. (ed.), Feb 2012, New York: Nova Science Publishers. 248 p.

Research output: Book/ReportEdited Book/AnthologyResearch

Motor Neuron Disease
Amyotrophic Lateral Sclerosis
Motor Neurons
Nerve Degeneration
Calcium

Mutant FUS induces endoplasmic reticulum stress in amyotrophic lateral sclerosis and interacts with protein disulfide-isomerase

Farg, M. A., Soo, K. Y., Walker, A. K., Pham, H., Orian, J., Horne, M. K., Warraich, S. T., Williams, K. L., Blair, I. P. & Atkin, J. D., Dec 2012, In : Neurobiology of Aging. 33, 12, p. 2855-2868 14 p.

Research output: Contribution to journalArticleResearchpeer-review

Protein Disulfide-Isomerases
Endoplasmic Reticulum Stress
Amyotrophic Lateral Sclerosis
Sarcoma
Motor Neurons

Preface

Turner, B. J. & Atkin, J. B., Feb 2012, Motor neuron diseases: causes, classification and treatments. Turner, B. J. & Atkin, J. B. (eds.). Hauppauge, NY: Nova Science Publishers, p. vii-xii 6 p.

Research output: Chapter in Book/Report/Conference proceedingForeword/postscript/introductionResearchpeer-review

2011

Mechanisms of neuroprotection by protein disulphide isomerase in amyotrophic lateral sclerosis

Walker, A. K. & Atkin, J. D., Feb 2011, In : Neurology Research International. 2011, p. 1-7 7 p., 317340.

Research output: Contribution to journalReview articleResearchpeer-review

Open Access
File
Protein Disulfide-Isomerases
Amyotrophic Lateral Sclerosis
Endoplasmic Reticulum Stress
Genetic Models
Motor Neurons

Molecular motor proteins and amyotrophic lateral sclerosis

Soo, K. Y., Farg, M. & Atkin, J. D., Dec 2011, In : International Journal of Molecular Sciences. 12, 12, p. 9057-9082 26 p.

Research output: Contribution to journalReview articleResearchpeer-review

Open Access
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Molecular Motor Proteins
Dyneins
Amyotrophic Lateral Sclerosis
Neurons
Motor Neurons

Stress signaling from the endoplasmic reticulum: A central player in the pathogenesis of amyotrophic lateral sclerosis

Walker, A. K. & Atkin, J. D., Sep 2011, In : IUBMB Life. 63, 9, p. 754-763 10 p.

Research output: Contribution to journalReview articleResearchpeer-review

Endoplasmic Reticulum Stress
Amyotrophic Lateral Sclerosis
Unfolded Protein Response
Proteins
Endoplasmic Reticulum
2010

Multifaceted deaths orchestrated by mitochondria in neurones

Nagley, P., Higgins, G. C., Atkin, J. D. & Beart, P. M., Jan 2010, In : Biochimica et Biophysica Acta - Molecular Basis of Disease. 1802, 1, p. 167-185 19 p.

Research output: Contribution to journalReview articleResearchpeer-review

Mitochondria
Neurons
Cell Death
Necrosis
Apoptosis

Protein disulphide isomerase protects against protein aggregation and is S-nitrosylated in amyotrophic lateral sclerosis

Walker, A. K., Farg, M. A., Bye, C. R., McLean, C. A., Horne, M. K. & Atkin, J. D., Jan 2010, In : Brain. 133, 1, p. 105-116 12 p.

Research output: Contribution to journalArticleResearchpeer-review

Protein Disulfide-Isomerases
Amyotrophic Lateral Sclerosis
Endoplasmic Reticulum Stress
Proteins
Motor Neurons

Serum matrix metalloproteinase-9 activity is dysregulated with disease progression in the mutant SOD1 transgenic mice

Soon, C. P. W., Crouch, P. J., Turner, B. J., McLean, C. A., Laughton, K. M., Atkin, J. D., Masters, C. L., White, A. R. & Li, Q. X., Apr 2010, In : Neuromuscular Disorders. 20, 4, p. 260-266 7 p.

Research output: Contribution to journalArticleResearchpeer-review

Matrix Metalloproteinase 9
Transgenic Mice
Disease Progression
Serum
Amyotrophic Lateral Sclerosis