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Research Outputs

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 conferenceAbstract

Article

ALS-associated mutant FUS inhibits macroautophagy which is restored by overexpression of Rab1

Soo, K. Y., Sultana, J., King, A. E., Atkinson, R. A. K., Warraich, S. T., Sundaramoorthy, V., Blair, I., Farg, M. A. & Atkin, J. D., 21 Dec 2015, In : Cell Death Discovery. 1, 1, p. 1-12 12 p., 15030.

Research output: Contribution to journalArticle

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28 Citations (Scopus)
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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 journalArticle

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77 Citations (Scopus)
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Amyotrophic lateral sclerosis-linked UBQLN2 mutants inhibit endoplasmic reticulum to Golgi transport, leading to Golgi fragmentation and ER stress

Halloran, M., Ragagnin, A. M. G., Vidal, M., Parakh, S., Yang, S., Heng, B., Grima, N., Shahheydari, H., Soo, K-Y., Blair, I., Guillemin, G. J., Sundaramoorthy, V. & Atkin, J. D., 4 Dec 2019, In : Cellular and Molecular Life Sciences. 15 p.

Research output: Contribution to journalArticle

12 Citations (Scopus)

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 journalArticle

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 journalArticle

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27 Citations (Scopus)
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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 journalArticle

10 Citations (Scopus)

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 journalArticle

211 Citations (Scopus)

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 journalArticle

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8 Citations (Scopus)
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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 journalArticle

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6 Citations (Scopus)
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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 others, Graff-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 journalArticle

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78 Citations (Scopus)
15 Downloads (Pure)

CYLD is a causative gene for frontotemporal dementia - amyotrophic lateral sclerosis

Dobson-Stone, C., Hallupp, M., Shahheydari, H., Ragagnin, A. M. G., Chatterton, Z., Carew-Jones, F., Shepherd, C. E., Stefen, H., Paric, E., Fath, T., Thompson, E. M., Blumbergs, P., Short, C. L., Field, C. D., Panegyres, P. K., Hecker, J., Nicholson, G., Shaw, A. D., Fullerton, J. M., Luty, A. A. & 19 others, Schofield, P. R., Brooks, W. S., Rajan, N., Bennett, M. F., Bahlo, M., Shankaracharya, Landers, J. E., Piguet, O., Hodges, J. R., Halliday, G. M., Topp, S. D., Smith, B. N., Shaw, C. E., McCann, E., Fifita, J. A., Williams, K. L., Atkin, J. D., Blair, I. P. & Kwok, J. B., 1 Mar 2020, In : Brain : a journal of neurology. 143, 3, p. 783-799 17 p.

Research output: Contribution to journalArticle

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 journalArticle

35 Citations (Scopus)

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 journalArticle

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48 Citations (Scopus)
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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 journalArticle

1 Citation (Scopus)

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 journalArticle

255 Citations (Scopus)

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 journalArticle

5 Citations (Scopus)

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 journalArticle

37 Citations (Scopus)

Genetic and immunopathological analysis of CHCHD10 in Australian amyotrophic lateral sclerosis and frontotemporal dementia and transgenic TDP-43 mice

McCann, E. P., Fifita, J. A., Grima, N., Galper, J., Mehta, P., Freckleton, S. E., Zhang, K. Y., Henden, L., Hogan, A. L., Chan Moi Fat, S., Wu, S. S. L., Jagaraj, C. J., Berning, B. A., Williams, K. L., Twine, N. A., Bauer, D., Piguet, O., Hodges, J., Kwok, J. B. J., Halliday, G. M. & 7 others, Kiernan, M. C., Atkin, J., Rowe, D. B., Nicholson, G. A., Walker, A. K., Blair, I. P. & Yang, S., 1 Feb 2020, In : Journal of Neurology, Neurosurgery and Psychiatry. 91, 2, p. 162-171 10 p.

Research output: Contribution to journalArticle

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 journalArticle

143 Citations (Scopus)
16 Citations (Scopus)

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 journalArticle

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 journalArticle

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2 Citations (Scopus)
8 Downloads (Pure)

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 journalArticle

4 Citations (Scopus)

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 journalArticle

60 Citations (Scopus)

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 journalArticle

61 Citations (Scopus)

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 journalArticle

40 Citations (Scopus)

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 journalArticle

Open Access
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16 Citations (Scopus)
9 Downloads (Pure)

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 journalArticle

8 Citations (Scopus)
Open Access
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37 Citations (Scopus)
7 Downloads (Pure)

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 others, Molloy, M. P. & Chung, R. S., Jan 2018, In : Cellular and Molecular Life Sciences. 75, 2, p. 335–354 20 p.

Research output: Contribution to journalArticle

6 Citations (Scopus)

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., 15 Mar 2015, In : Human molecular genetics. 24, 6, p. 1655-1669 15 p., ddu578.

Research output: Contribution to journalArticle

18 Citations (Scopus)

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 journalArticle

44 Citations (Scopus)

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 journalArticle

3 Citations (Scopus)

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 journalArticle

114 Citations (Scopus)

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 journalArticle

Open Access
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15 Citations (Scopus)
10 Downloads (Pure)

Purification and characterization of chimeric human IgA1 and IgA2 expressed in COS and Chinese hamster ovary cells

Morton, H. C., Atkin, J. D., Owens, R. J. & Woof, J. M., 1993, In : Journal of Immunology. 151, 9, p. 4743-4752 10 p.

Research output: Contribution to journalArticle

66 Citations (Scopus)

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 journalArticle

42 Citations (Scopus)

Recognition of an octapeptide sequence by multiple Galα(1,3)Gal-binding proteins

Vaughan, H. A., Oldenburg, K. R., Gallop, M. A., Atkin, J. D., McKenzie, I. F. C. & Sandrin, M. S., Feb 1996, In : Xenotransplantation. 3, 1 PART 1, p. 18-23 6 p.

Research output: Contribution to journalArticle

33 Citations (Scopus)

Recombinant human antibodies: linkage of an Fab fragment from a combinatorial library to an Fc fragment for expression in mammalian cell culture

Bender, E., Woof, J. M., Atkin, J. D., Barker, M. D., Bebbington, C. R. & Burton, D. R., 1993, In : Human Antibodies. 4, 2, p. 74-79 6 p.

Research output: Contribution to journalArticle

45 Citations (Scopus)

Recruitment of mitochondria into apoptotic signaling correlates with the presence of inclusions formed by amyotrophic lateral sclerosis-associated SOD1 mutations

Soo, K. Y., Atkin, J. D., Horne, M. K. & Nagley, P., Feb 2009, In : Journal of Neurochemistry. 108, 3, p. 578-590 13 p.

Research output: Contribution to journalArticle

24 Citations (Scopus)

Riluzole does not ameliorate disease caused by cytoplasmic TDP-43 in a mouse model of amyotrophic lateral sclerosis

Wright, A. L., Le, S., Mehta, P., Gatta, P. D., Berning, B. A., Jacobs, K. R., Gul, H., Gil, R. S., Hedl, T. J., Riddell, W. R., Watson, O., Keating, S. S., Chung, R. S., Atkin, J. D., Lee, A., Shi, B., Blizzard, C. A., Morsch, M. & Walker, A. K., 29 Aug 2019, (In preparation) In : bioRxiv.

Research output: Contribution to journalArticle

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 journalArticle

18 Citations (Scopus)

Sex-specific behavioural effects of environmental enrichment in a transgenic mouse model of amyotrophic lateral sclerosis

Stam, N. C., Nithianantharajah, J., Howard, M. L., Atkin, J. D., Cheema, S. S. & Hannan, A. J., Aug 2008, In : European Journal of Neuroscience. 28, 4, p. 717-723 7 p.

Research output: Contribution to journalArticle

41 Citations (Scopus)

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 journalArticle

5 Citations (Scopus)

Strategies to overcome the anti-Galα(1-3)Gal reaction in xenotransplantation

McKenzie, I. F. C., Osman, N., Cohney, S., Vaughan, H. A., Patton, K., Mouhtouris, E., Atkin, J. D., Elliott, E., Fodor, W. L., Squinto, S. P., Burton, D., Gallop, M. A., Oldenburg, K. R. & Sandrin, M. S., 1996, In : Transplantation Proceedings. 28, 2, p. 537 1 p.

Research output: Contribution to journalArticle

12 Citations (Scopus)

Structural Requirements for Assembly of Dimeric IgA Probed by Site-Directed Mutagenesis of J Chain and a Cysteine Residue of the α-Chain CH2 Domain

Krugmann, S., Pleass, R. J., Atkin, J. D. & Woof, J. M., 1997, In : Journal of Immunology. 159, 1, p. 244-249 6 p.

Research output: Contribution to journalArticle

40 Citations (Scopus)

Structural requirements for the interaction of human IgA with the human polymeric Ig receptor

Lewis, M. J., Pleass, R. J., Batten, M. R., Atkin, J. D. & Woof, J. M., 15 Nov 2005, In : Journal of Immunology. 175, 10, p. 6694-6701 8 p.

Research output: Contribution to journalArticle

33 Citations (Scopus)