List of works - Julie D Atkin

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

scientific article

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

scientific article

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

scientific article published on 14 September 2015

Amyotrophic lateral sclerosis-linked UBQLN2 mutants inhibit endoplasmic reticulum to Golgi transport, leading to Golgi fragmentation and ER stress

scientific article published on 04 December 2019

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

scientific article published on 29 October 2015

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

scientific article published in December 2004

C9ORF72 expression and cellular localization over mouse development.

scientific article

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

scientific article

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

scientific article published on 10 August 2017

CCNF mutations in amyotrophic lateral sclerosis and frontotemporal dementia

scientific article published on 15 April 2016

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

scientific article published on 01 March 2020

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

scientific article

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

scientific article published in September 2017

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

scientific article published on 9 April 2015

Dysfunction of Optineurin in Amyotrophic Lateral Sclerosis and Glaucoma.

scientific article published on 23 May 2018

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

scientific article published on 26 September 2013

ER stress and UPR in familial amyotrophic lateral sclerosis.

scientific article

ERp57 is protective against mutant SOD1-induced cellular pathology in Amyotrophic Lateral Sclerosis.

scientific article published in February 2018

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

scientific article published on 18 February 2016

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

scientific article

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

scientific article published on 14 June 2013

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

scientific article published on 05 November 2019

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

scientific article

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

scientific article

Impaired NHEJ repair in amyotrophic lateral sclerosis is associated with TDP-43 mutations

scientific article published on 09 September 2020

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

scientific journal article

Is cytoplasmic FUS a feature of all ALS?

scientific article published on 01 September 2019

Mechanisms of neuroprotection by protein disulphide isomerase in amyotrophic lateral sclerosis

scientific article

Molecular motor proteins and amyotrophic lateral sclerosis

scientific article

Motor Neuron Abnormalities Correlate with Impaired Movement in Zebrafish that Express Mutant Superoxide Dismutase 1

scientific article published on 27 October 2018

Motor Neuron Susceptibility in ALS/FTD

scientific article published on 27 June 2019

Multifaceted deaths orchestrated by mitochondria in neurones.

scientific article published on 12 September 2009

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

scientific article

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

scientific article

Mutant SOD1 inhibits ER-Golgi transport in amyotrophic lateral sclerosis.

scientific article published on 13 November 2013

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

scientific article published on 14 December 2012

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

scientific article published on 13 April 2016

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

scientific article published on 21 May 2015

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

scientific article published on 29 August 2017

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

scientific article

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

scientific article published in May 2005

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

scientific article

Protein Folding Alterations in Amyotrophic Lateral Sclerosis.

scientific article published on 7 April 2016

Protein Quality Control and the Amyotrophic Lateral Sclerosis/Frontotemporal Dementia Continuum

scientific article

Protein disulphide isomerase (PDI) is protective against amyotrophic lateral sclerosis (ALS)-related mutant Fused in Sarcoma (FUS) in in vitro models

scientific article published on 02 September 2021

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

scientific article published on 01 January 2019

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

scientific article published on November 1993

Rab-dependent cellular trafficking and amyotrophic lateral sclerosis

scientific article published on 01 December 2018

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

scientific article published on 23 August 2015

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

scientific article published on April 1993

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

scientific article

Redox regulation in amyotrophic lateral sclerosis

scientific article

SOD1 Mutations Causing Familial Amyotrophic Lateral Sclerosis Induce Toxicity in Astrocytes: Evidence for Bystander Effects in a Continuum of Astrogliosis

scientific article

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

scientific article published on 25 January 2010

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

scientific article published on August 2008

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

scientific article published on 01 April 1996

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

scientific article published on 10 August 2011

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

scientific article published in November 2005

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

scientific article published on 8 May 2017

The Emerging Role of DNA Damage in the Pathogenesis of the C9orf72 Repeat Expansion in Amyotrophic Lateral Sclerosis

scientific article published on 12 October 2018

The Unfolded Protein Response and the Role of Protein Disulfide Isomerase in Neurodegeneration

scientific article

The complement factor C5a contributes to pathology in a rat model of amyotrophic lateral sclerosis

scientific article published on 01 December 2008

The microglial NLRP3 inflammasome is activated by amyotrophic lateral sclerosis proteins

scientific article published on 09 October 2019

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

scientific article

List of works by Julie D Atkin

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

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

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

Amyotrophic lateral sclerosis-linked UBQLN2 mutants inhibit endoplasmic reticulum to Golgi transport, leading to Golgi fragmentation and ER stress

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

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

C9ORF72 expression and cellular localization over mouse development.

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

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

CCNF mutations in amyotrophic lateral sclerosis and frontotemporal dementia

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

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

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

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

Dysfunction of Optineurin in Amyotrophic Lateral Sclerosis and Glaucoma.

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

ER stress and UPR in familial amyotrophic lateral sclerosis.

ERp57 is protective against mutant SOD1-induced cellular pathology in Amyotrophic Lateral Sclerosis.

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

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

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

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

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

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

Impaired NHEJ repair in amyotrophic lateral sclerosis is associated with TDP-43 mutations

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

Is cytoplasmic FUS a feature of all ALS?

Mechanisms of neuroprotection by protein disulphide isomerase in amyotrophic lateral sclerosis

Molecular motor proteins and amyotrophic lateral sclerosis

Motor Neuron Abnormalities Correlate with Impaired Movement in Zebrafish that Express Mutant Superoxide Dismutase 1

Motor Neuron Susceptibility in ALS/FTD

Multifaceted deaths orchestrated by mitochondria in neurones.

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

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

Mutant SOD1 inhibits ER-Golgi transport in amyotrophic lateral sclerosis.

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

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

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

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

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

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

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

Protein Folding Alterations in Amyotrophic Lateral Sclerosis.

Protein Quality Control and the Amyotrophic Lateral Sclerosis/Frontotemporal Dementia Continuum

Protein disulphide isomerase (PDI) is protective against amyotrophic lateral sclerosis (ALS)-related mutant Fused in Sarcoma (FUS) in in vitro models

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

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

Rab-dependent cellular trafficking and amyotrophic lateral sclerosis

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

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

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

Redox regulation in amyotrophic lateral sclerosis

SOD1 Mutations Causing Familial Amyotrophic Lateral Sclerosis Induce Toxicity in Astrocytes: Evidence for Bystander Effects in a Continuum of Astrogliosis

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

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

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

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

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

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

The Emerging Role of DNA Damage in the Pathogenesis of the C9orf72 Repeat Expansion in Amyotrophic Lateral Sclerosis

The Unfolded Protein Response and the Role of Protein Disulfide Isomerase in Neurodegeneration

The complement factor C5a contributes to pathology in a rat model of amyotrophic lateral sclerosis

The microglial NLRP3 inflammasome is activated by amyotrophic lateral sclerosis proteins

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