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Research Outputs 1993 2019

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

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

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

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

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

Brain β-amyloid accumulation in transgenic mice expressing mutant superoxide dismutase 1

Turner, B. J., Li, Q. X., Laughton, K. M., Masters, C. L., Lopes, E. C., Atkin, J. D. & Cheema, S. S., Dec 2004, In : Neurochemical Research. 29, 12, p. 2281-2286 6 p.

Research output: Contribution to journalArticleResearchpeer-review

Amyloid
Transgenic Mice
Superoxide Dismutase
Brain
Amyotrophic Lateral Sclerosis

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

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

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

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

Coupling ER stress to motor neuron degeneration in ALS

Turner, B. J. & Atkin, J. D., 2008, Motor neuron disease research progress. Mancini, R. L. (ed.). New York: Nova Science Publishers, p. 51-81 31 p.

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

Nerve Degeneration
Endoplasmic Reticulum Stress
Amyotrophic Lateral Sclerosis
Motor Neurons
Unfolded Protein Response

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

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

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

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

Endoplasmic reticulum stress and induction of the unfolded protein response in human sporadic amyotrophic lateral sclerosis

Atkin, J. D., Farg, M. A., Walker, A. K., McLean, C., Tomas, D. & Horne, M. K., Jun 2008, In : Neurobiology of Disease. 30, 3, p. 400-407 8 p.

Research output: Contribution to journalArticleResearchpeer-review

Unfolded Protein Response
Endoplasmic Reticulum Stress
Amyotrophic Lateral Sclerosis
Rodentia
Protein Disulfide-Isomerases

Endoplasmic reticulum stress and protein misfolding in amyotrophic lateral sclerosis

Walker, A. K., Turner, B. J. & Atkin, J. D., 2009, Protein Misfolding Disorders: A Trip into the ER. Claudio, H. (ed.). Chile: Bentham Science Publishers Ltd., p. 56-76 21 p.

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

Endoplasmic Reticulum Stress
Amyotrophic Lateral Sclerosis
Sclerosis
Heat-Shock Proteins
Unfolded Protein Response

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

ER stress and UPR in familial amyotrophic lateral sclerosis

Turner, B. J. & Atkin, J. D., Feb 2006, In : Current Molecular Medicine. 6, 1, p. 79-86 8 p.

Research output: Contribution to journalReview articleResearchpeer-review

Unfolded Protein Response
Endoplasmic Reticulum Stress
Heat-Shock Proteins
Endoplasmic Reticulum
Proteins

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

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

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

Impaired extracellular secretion of mutant superoxide dismutase 1 associates with neurotoxicity in familial amyotrophic lateral sclerosis

Turner, B. J., Atkin, J. D., Farg, M. A., Da, W. Z., Rembach, A., Lopes, E. C., Patch, J. D., Hill, A. F. & Cheema, S. S., 5 Jan 2005, In : Journal of Neuroscience. 25, 1, p. 108-117 10 p.

Research output: Contribution to journalArticleResearchpeer-review

Brefeldin A
Inclusion Bodies
Amyotrophic Lateral Sclerosis
Transgenic Rats
Secretory Pathway

Increased number and differentiation of neural precursor cells in the brainstem of superoxide dismutase 1G93A G1H transgenic mouse model of amyotrophic lateral sclerosis

Juan, L., Dawei, Z. & Julie, A. D., Mar 2007, In : Neurological Research. 29, 2, p. 204-209 6 p.

Research output: Contribution to journalArticleResearchpeer-review

Amyotrophic Lateral Sclerosis
Transgenic Mice
Brain Stem
Superoxide Dismutase
Glial Fibrillary Acidic Protein

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

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

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

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
File
Molecular Motor Proteins
Dyneins
Amyotrophic Lateral Sclerosis
Neurons
Motor Neurons

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 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

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

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

Mutagenesis of J chain residues critical for IgA dimer assembly

Krugmann, S., Pleass, R. J., Atkin, J. D. & Woof, J. M., 1997, In : Biochemical Society Transactions. 25, 2, p. 323S 1 p.

Research output: Contribution to journalArticleResearchpeer-review

Mutagenesis of the human IgA1 heavy chain tailpiece that prevents dimer assembly

Atkin, J. D., Pleass, R. J., Owens, R. J. & Woof, J. M., 1 Jul 1996, In : Journal of Immunology. 157, 1, p. 156-159 4 p.

Research output: Contribution to journalArticleResearchpeer-review

Mutagenesis
Immunoglobulin A
Protein Engineering
Terminator Codon
Dimerization

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

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

Mutations in a new ALS/FTD gene, CCNF, uncovers new mechanisms of Lys48-ubiquitylation regulation by the Skp-Cul-F-Box(Cyclin F) (SCF(CyclinF)) E3 ligase complex

Lee, A., Rayner, S., Gwee, S., Sundaramoorthy, V., Morsch, M., Radford, R., Yang, S., Williams, K., Hogan, A., Don, E., Cole, N., Blair, I., Atkin, J., Molloy, M. & Chung, R., 7 Dec 2016, In : Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration. 17, Supplement 1, p. 163 1 p., P130.

Research output: Contribution to journalMeeting abstractResearchpeer-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

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

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

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

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., Morsch, M., Hogan, A., Don, E., Williams, K., Yerbury, J. J., Blair, I., Atkin, J. D., Molloy, M. P. & Chung, R. S., 8 Feb 2019. 1 p.

Research output: Contribution to conferenceAbstractResearchpeer-review

Cyclins
Ubiquitination
Autophagy
Mutation
Genes

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

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

Properties of slow- and fast-twitch muscle fibres in a mouse model of amyotrophic lateral sclerosis

Atkin, J. D., Scott, R. L., West, J. M., Lopes, E., Quah, A. K. J. & Cheema, S. S., May 2005, In : Neuromuscular Disorders. 15, 5, p. 377-388 12 p.

Research output: Contribution to journalArticleResearchpeer-review

Slow-Twitch Muscle Fibers
Fast-Twitch Muscle Fibers
Amyotrophic Lateral Sclerosis
Muscles
Neuromuscular Junction

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

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

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 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

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