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The deubiquitinase function of ataxin-3 and its role in the pathogenesis of Machado-Joseph disease and other diseases

Anastasiya Potapenko, Jennilee M. Davidson, Albert Lee, Angela S. Laird

Biochem J (2024) 481 (6): 461–480.

https://doi.org/10.1042/BCJ20240017

Published: 18 March 2024

... in MJD and also appears to be involved in cancer. As aberrant deubiquitination has been linked to both neurodegeneration and cancer, a comprehensive understanding of ataxin-3's DUB function is important for elucidating potential therapeutic targets in these complex conditions. In this review, we aim...

View article titled, The deubiquitinase function of ataxin-3 and its role in the pathogenesis of Machado-Joseph disease and other diseases

Articles

Proximity labelling reveals effects of disease-causing mutation on the DNAJC5/cysteine string protein α interactome

Eleanor Barker, Amy E. Milburn, Nordine Helassa, Dean E. Hammond, Natalia Sanchez-Soriano, Alan Morgan, Jeff W. Barclay

Biochem J (2024) 481 (3): 141–160.

https://doi.org/10.1042/BCJ20230319

Published: 29 January 2024

... and mice similarly result in neurodegeneration, CSP is evidently an evolutionarily conserved neuroprotective protein. However, the client proteins that CSP chaperones to prevent neurodegeneration remain unclear. Traditional methods for identifying protein–protein interactions, such as yeast 2-hybrid...

View article titled, Proximity labelling reveals effects of disease-causing mutation on the DNAJC5/cysteine string protein α interactome

Includes: Supplementary data

Articles

Genetic modulation of CWD prion propagation in cervid PrP Drosophila

Alana M. Thackray, Erin E. McNulty, Amy V. Nalls, Alzbeta Cardova, Linh Tran, Glenn Telling, Sylvie L. Benestad, Sabine Gilch, Candace K. Mathiason, Raymond Bujdoso

Biochem J (2023) 480 (19): 1485–1501.

https://doi.org/10.1042/BCJ20230247

Published: 04 October 2023

... a transformative agreement with JISC. cervid CWD Drosophila melanogaster infectivity neurodegeneration prion Prion diseases are fatal transmissible neurodegenerative conditions of mammalian species that include Creutzfeldt–Jakob disease (CJD) of humans, bovine spongiform encephalopathy (BSE...

View article titled, Genetic modulation of CWD prion propagation in cervid PrP Drosophila

Articles

The complex interplay between autophagy and cell death pathways

In Collection

Cell death and survival

Christina Ploumi, Margarita-Elena Papandreou, Nektarios Tavernarakis

Biochem J (2022) 479 (1): 75–90.

https://doi.org/10.1042/BCJ20210450

Published: 14 January 2022

... this cross-talk is disrupted, in the context of disease. apoptosis autophagic cell death autophagy cancer necroptosis neurodegeneration Correspondence: Nektarios Tavernarakis ( [email protected] ) 14 11 2021 21 12 2021 23 12 2021 © 2022 The Author(s...

View article titled, The complex interplay between autophagy and cell death pathways

Articles

From structure to ætiology: a new window on the biology of leucine-rich repeat kinase 2 and Parkinson's disease

Susanne Herbst, Patrick A. Lewis

Biochem J (2021) 478 (14): 2945–2951.

https://doi.org/10.1042/BCJ20210383

Published: 30 July 2021

... for this article was enabled by the participation of University College London in an all-inclusive Read & Publish pilot with Portland Press and the Biochemical Society under a transformative agreement with JISC. leucine-rich repeat kinase neurodegeneration Parkinson's disease Leucine-rich...

View article titled, From structure to ætiology: a new window on the biology of leucine-rich repeat kinase 2 and Parkinson's disease

Articles

Mechanistic roles for altered O -GlcNAcylation in neurodegenerative disorders

Aaron T. Balana, Matthew R. Pratt

Biochem J (2021) 478 (14): 2733–2758.

https://doi.org/10.1042/BCJ20200609

Published: 23 July 2021

... proposed as a potential strategy to slow down the progression of neurodegeneration. Through the development and application of tools that allow dissection of the mechanistic roles of this PTM, there is now a growing body of evidence that O -GlcNAc influences a variety of important neurodegeneration...

View article titled, Mechanistic roles for altered O -GlcNAcylation in neurodegenerative disorders

Articles

Understanding amphisomes

Dhasarathan Ganesan, Qian Cai

Biochem J (2021) 478 (10): 1959–1976.

https://doi.org/10.1042/BCJ20200917

Published: 28 May 2021

... amphisome autophagy late endosome neurodegeneration neurotrophic signaling retrograde axonal transport Macroautophagy, hereafter referred to as autophagy, is a major cytosolic degradative system involving sequestration of damaged cellular components and dysfunctional organelles within...

View article titled, Understanding amphisomes

Articles

Transcriptional signature of prion-induced neurotoxicity in a Drosophila model of transmissible mammalian prion disease

Alana M. Thackray, Brian Lam, Anisa Shahira Binti Ab Razak, Giles Yeo, Raymond Bujdoso

Biochem J (2020) 477 (4): 833–852.

https://doi.org/10.1042/BCJ20190872

Published: 28 February 2020

... with Portland Press and the Biochemical Society under a transformative agreement with JISC. Drosophila melanogaster neurodegeneration prion transcriptomics Protein misfolding neurodegenerative diseases are invariably fatal conditions that include Alzheimer's disease, Huntington's disease...

View article titled, Transcriptional signature of prion-induced neurotoxicity in a Drosophila model of transmissible mammalian prion disease

Includes: Supplementary data

Articles

Astroglial contribution to tau-dependent neurodegeneration

Marta Sidoryk-Węgrzynowicz, Lidia Strużyńska

Biochem J (2019) 476 (22): 3493–3504.

https://doi.org/10.1042/BCJ20190506

Published: 27 November 2019

...-dependent neurodegeneration, where astrocytes lose key molecules involved in regulation of glutamate/glutamine homeostasis, neuronal survival and synaptogenesis. Astrocytic dysfunction in tauopathy appears to precede neurodegeneration and overt tau neuropathology such as phosphorylation, aggregation...

View article titled, Astroglial contribution to tau-dependent neurodegeneration

Articles

Cysteine-rich granulin-3 rapidly promotes amyloid-β fibrils in both redox states

Anukool A. Bhopatkar, Gaurav Ghag, Lauren M. Wolf, Dexter N. Dean, Melissa A. Moss, Vijayaraghavan Rangachari

Biochem J (2019) 476 (5): 859–873.

https://doi.org/10.1042/BCJ20180916

Published: 12 March 2019

...-rich proteins granulin neurodegeneration progranulin Granulins (GRNs 1–7) are a family of small unique cysteine-rich proteins that are proteolytically cleaved from the precursor protein, progranulin (PGRN) ( Supplementary Figure S1A ) [ 1 ]. All seven GRNs (GRNs 1–7) are ∼6 kDa in size...

View article titled, Cysteine-rich granulin-3 rapidly promotes amyloid-β fibrils in both redox states

Includes: Supplementary data

Articles

An Aβ42 variant that inhibits intra- and extracellular amyloid aggregation and enhances cell viability

Ofek Oren, Victor Banerjee, Ran Taube, Niv Papo

Biochem J (2018) 475 (19): 3087–3103.

https://doi.org/10.1042/BCJ20180247

Published: 10 October 2018

... ( [email protected] ) or Niv Papo ( [email protected] ) 26 3 2018 2 9 2018 7 9 2018 13 9 2018 © 2018 The Author(s). Published by Portland Press Limited on behalf of the Biochemical Society 2018 Aβ42 peptide Alzheimer's disease amyloids neurodegeneration neuronal cell...

View article titled, An Aβ42 variant that inhibits intra- and extracellular amyloid aggregation and enhances cell viability

Includes: Supplementary data

Articles

RNA-binding proteins with prion-like domains in health and disease

Alice Ford Harrison, James Shorter

Biochem J (2017) 474 (8): 1417–1438.

https://doi.org/10.1042/BCJ20160499

Published: 07 April 2017

... RBPs with PrLDs. We also highlight the potential of protein disaggregases, including Hsp104, as a therapeutic strategy to combat the aberrant phase transitions of RBPs with PrLDs that likely underpin neurodegeneration. Correspondence: James Shorter ( [email protected] ) 9 1 2017...

View article titled, RNA-binding proteins with prion-like domains in health and disease

Articles

Astrocytic transporters in Alzheimer's disease

Chris Ugbode, Yuhan Hu, Benjamin Whalley, Chris Peers, Marcus Rattray, Mark L. Dallas

Biochem J (2017) 474 (3): 333–355.

https://doi.org/10.1042/BCJ20160505

Published: 20 January 2017

... transport system covers a diverse array of proteins involved in metabolic support, neurotransmission and synaptic architecture. Therefore, specific targeting of individual transporter families has the potential to suppress neurodegeneration, a characteristic hallmark of AD. A small number of the 400...

View article titled, Astrocytic transporters in Alzheimer's disease

Articles

Systematic approaches to identify E3 ligase substrates

Mary Iconomou, Darren N. Saunders

Biochem J (2016) 473 (22): 4083–4101.

https://doi.org/10.1042/BCJ20160719

Published: 10 November 2016

... article published by Portland Press Limited on behalf of the Biochemical Society and distributed under the Creative Commons Attribution License 4.0 (CC BY-NC-ND) . cancer functional genomics neurodegeneration proteomics proteostasis ubiquitin Conjugation of the small protein modifier...

View article titled, Systematic approaches to identify E3 ligase substrates

Articles

Plasma metabolic profile delineates roles for neurodegeneration, pro-inflammatory damage and mitochondrial dysfunction in the FMR1 premutation

Cecilia Giulivi, Eleonora Napoli, Flora Tassone, Julian Halmai, Randi Hagerman

Biochem J (2016) 473 (21): 3871–3888.

https://doi.org/10.1042/BCJ20160585

Published: 27 October 2016

... with increases in lactate levels and altered Krebs' intermediates, neurotransmitters, markers of neurodegeneration and increases in oxidative stress-mediated damage to biomolecules. The number of CGG repeats correlated with a subset of plasma metabolites, which are implicated not only in mitochondrial disorders...

View article titled, Plasma metabolic profile delineates roles for neurodegeneration, pro-inflammatory damage and mitochondrial dysfunction in the FMR1 premutation

Includes: Supplementary data

Articles

High-affinity Anticalins with aggregation-blocking activity directed against the Alzheimer β-amyloid peptide

Sabine Rauth, Dominik Hinz, Michael Börger, Markus Uhrig, Manuel Mayhaus, Matthias Riemenschneider, Arne Skerra

Biochem J (2016) 473 (11): 1563–1578.

https://doi.org/10.1042/BCJ20160114

Published: 27 May 2016

...) and alternative drug candidates compared with current biopharmaceutical treatments. Aβ peptide lipocalin neurodegeneration protein engineering Alzheimer's disease (AD) is the most prevalent form of dementia, with 10% of the human population older than 65 years and 40% older than 85 years affected...

View article titled, High-affinity Anticalins with aggregation-blocking activity directed against the Alzheimer β-amyloid peptide

Includes: Supplementary data

Articles

The function of orthologues of the human Parkinson's disease gene LRRK2 across species: implications for disease modelling in preclinical research

Rebekah G. Langston, Iakov N. Rudenko, Mark R. Cookson

Biochem J (2016) 473 (3): 221–232.

https://doi.org/10.1042/BJ20150985

Published: 25 January 2016

... GTPase kinase LRRK2 homologue neurodegeneration preclinical research The cause of most cases of PD is not known, leading to the description as a sporadic or idiopathic disease. However, in recent years it has been increasingly appreciated that monogenic forms of PD exist and that cloning...

View article titled, The function of orthologues of the human Parkinson's disease gene LRRK2 across species: implications for disease modelling in preclinical research

Articles

DJ-1 interacts with RACK1 and protects neurons from oxidative-stress-induced apoptosis

Jun Ma, Rong Wu, Qiang Zhang, Jun-bing Wu, Jizhong Lou, Zheng Zheng, Jian-qing Ding, Zengqiang Yuan

Biochem J (2014) 462 (3): 489–497.

https://doi.org/10.1042/BJ20140235

Published: 22 August 2014

... disease models. apoptosis DJ-1 neurodegeneration neuron receptor of activated C kinase 1 (RACK1) Animals were maintained in the Animal Care Facility at our institute, and all experiments involving animals were approved by Institutional Animal Care and Use Committee at the Institute...

View article titled, DJ-1 interacts with RACK1 and protects neurons from oxidative-stress-induced apoptosis

Articles

Impaired selenoprotein expression in brain triggers striatal neuronal loss leading to co-ordination defects in mice

Sandra Seeher, Bradley A. Carlson, Angela C. Miniard, Eva K. Wirth, Yassin Mahdi, Dolph L. Hatfield, Donna M. Driscoll, Ulrich Schweizer

Biochem J (2014) 462 (1): 67–75.

https://doi.org/10.1042/BJ20140423

Published: 24 July 2014

... seizures and neurodegeneration [ 15 ]. Neurons account for most of the SeP expression in the mouse brain [ 16 ]. This finding was substantiated when SeP biosynthesis was inactivated selectively in neurons through the conditional inactivation of tRNA [Ser]Sec [ 17 ]. Specifically, PV (parvalbumin...

View article titled, Impaired selenoprotein expression in brain triggers striatal neuronal loss leading to co-ordination defects in mice

Includes: Multimedia, Supplementary data

Articles

Casein kinase: the triple meaning of a misnomer

Andrea Venerando, Maria Ruzzene, Lorenzo A. Pinna

Biochem J (2014) 460 (2): 141–156.

https://doi.org/10.1042/BJ20140178

Published: 13 May 2014

... 20C (Fam20C) genuine casein kinase (G-CK) neurodegeneration In the second half of the 19th Century, the findings of Ulaf Hammarsten [ 1 ] that phosphorus was present in the milk protein casein, in addition to carbon, nitrogen, hydrogen, oxygen and sulfur, came as a surprise. Henceforth...

View article titled, Casein kinase: the triple meaning of a misnomer

Includes: Supplementary data

Articles

The metalloproteases meprin α and meprin β: unique enzymes in inflammation, neurodegeneration, cancer and fibrosis

Claudia Broder, Christoph Becker-Pauly

Biochem J (2013) 450 (2): 253–264.

https://doi.org/10.1042/BJ20121751

Published: 15 February 2013

... fibrosis inflammation meprin metalloprotease neurodegeneration proteomics Proteolysis is an irreversible post-translational modification that affects every single cell of an organism. Over the last decades proteolytic enzymes have been identified to be master switches in the regulation...

View article titled, The metalloproteases meprin α and meprin β: unique enzymes in inflammation, neurodegeneration, cancer and fibrosis

Includes: Multimedia, Supplementary data

Articles

Peptidylarginine deiminase modulates the physiological roles of enolase via citrullination: links between altered multifunction of enolase and neurodegenerative diseases

Byungki Jang, Yong-Chul Jeon, Jin-Kyu Choi, Mira Park, Jae-Il Kim, Akihito Ishigami, Naoki Maruyama, Richard I. Carp, Yong-Sun Kim, Eun-Kyoung Choi

Biochem J (2012) 445 (2): 183–192.

https://doi.org/10.1042/BJ20120025

Published: 27 June 2012

... Society 2012 calpain citrullination enolase neurodegeneration peptidylarginine deiminase (PAD) plasminogen Citrullination converts positively charged arginine residues into neutral citrulline residues; this process is a post-translational modification of proteins that is regulated...

View article titled, Peptidylarginine deiminase modulates the physiological roles of enolase via citrullination: links between altered multifunction of enolase and neurodegenerative diseases

Articles

Autophagy, mitochondria and oxidative stress: cross-talk and redox signalling

Jisun Lee, Samantha Giordano, Jianhua Zhang

Biochem J (2012) 441 (2): 523–540.

https://doi.org/10.1042/BJ20111451

Published: 21 December 2011

... of the Creative Commons Attribution Non-Commercial Licence ( http://creativecommons.org/licenses/by-nc/2.5/ ) which permits unrestricted non-commercial use, distribution and reproduction in any medium, provided the original work is properly cited. autophagy mitochondrion neurodegeneration nitrative...

View article titled, Autophagy, mitochondria and oxidative stress: cross-talk and redox signalling

Articles

Inhibition of polyglutamine-mediated proteotoxicity by Astragalus membranaceus polysaccharide through the DAF-16/FOXO transcription factor in Caenorhabditis elegans

Hanrui Zhang, Ni Pan, Siqin Xiong, Shenglong Zou, Haifeng Li, Lingyun Xiao, Zhijian Cao, Alan Tunnacliffe, Zebo Huang

Biochem J (2012) 441 (1): 417–424.

https://doi.org/10.1042/BJ20110621

Published: 14 December 2011

... be addressed (email [email protected] ). 6 4 2011 15 8 2011 5 9 2011 5 9 2011 © The Authors Journal compilation © 2012 Biochemical Society 2012 Astragalus membranaceus lifespan neurodegeneration polyglutamine polysaccharide proteostasis To ensure normal...

View article titled, Inhibition of polyglutamine-mediated proteotoxicity by Astragalus membranaceus polysaccharide through the DAF-16/FOXO transcription factor in Caenorhabditis elegans

Includes: Supplementary data

Articles

TTBK2 kinase substrate specificity and the impact of spinocerebellar-ataxia-causing mutations on expression, activity, localization and development

Michale Bouskila, Noor Esoof, Laurie Gay, Emily H. Fang, Maria Deak, Michael J. Begley, Lewis C. Cantley, Alan Prescott, Kate G. Storey, Dario R. Alessi

Biochem J (2011) 437 (1): 157–167.

https://doi.org/10.1042/BJ20110276

Published: 14 June 2011

... disorder neurodegeneration protein kinase signal transduction tau tubulin kinase peptide substrate (TTBKtide) Previous studies have revealed that mutations within TTBK2 (tau tubulin kinase 2) cause a serious autosomal dominant inherited movement disorder termed SCA11 (spinocerebellar ataxia...

View article titled, TTBK2 kinase substrate specificity and the impact of spinocerebellar-ataxia-causing mutations on expression, activity, localization and development

Includes: Supplementary data

Articles

Rescuing neurons in prion disease

Nicholas C. Verity, Giovanna R. Mallucci

Biochem J (2011) 433 (1): 19–29.

https://doi.org/10.1042/BJ20101323

Published: 15 December 2010

..., Huntington's and Parkinson's diseases, and the rarer prion diseases, are separate entities clinically but have common features, including aggregates of misfolded proteins and varying patterns of neurodegeneration. A key barrier to effective treatment is that patients present clinically with advanced...

View article titled, Rescuing neurons in prion disease

Includes: Supplementary data

Articles

Evidence of mitochondrial dysfunction in fragile X-associated tremor/ataxia syndrome

Catherine Ross-Inta, Alicja Omanska-Klusek, Sarah Wong, Cedrick Barrow, Dolores Garcia-Arocena, Christine Iwahashi, Elizabeth Berry-Kravis, Randi J. Hagerman, Paul J. Hagerman, Cecilia Giulivi

Biochem J (2010) 429 (3): 545–552.

https://doi.org/10.1042/BJ20091960

Published: 14 July 2010

... tremor/ataxia syndrome (FXTAS) mitochondrion neurodegeneration oxidative stress trinucleotide repeat expansion unfolded protein response FXTAS (fragile X-associated tremor/ataxia syndrome) is a late-onset neurodegenerative disorder [ 1 – 5 ] that affects individuals who are carriers...

View article titled, Evidence of mitochondrial dysfunction in fragile X-associated tremor/ataxia syndrome

Includes: Supplementary data

Articles

Role of nitric oxide-mediated glutathionylation in neuronal function: potential regulation of energy utilization

Li-Peng Yap, Jerome V. Garcia, Derick S. Han, Enrique Cadenas

Biochem J (2010) 428 (1): 85–93.

https://doi.org/10.1042/BJ20100164

Published: 28 April 2010

... 2010 9 3 2010 © The Authors Journal compilation © 2010 Biochemical Society 2010 (3) Excessive generation of NO • in neuroinflammation, excitotoxicity, and during aging and age-related neurodegeneration entails the up-regulation of the inducible (iNOS) and neuronal (nNOS...

View article titled, Role of nitric oxide-mediated glutathionylation in neuronal function: potential regulation of energy utilization

Articles

Regulation and function of selenoproteins in human disease

Frederick P. Bellinger, Arjun V. Raman, Mariclair A. Reeves, Marla J. Berry

Biochem J (2009) 422 (1): 11–22.

https://doi.org/10.1042/BJ20090219

Published: 29 July 2009

... neurodegeneration selenium selenocysteine selenoprotein thyroid hormone Eukaryotic selenoprotein genes require a SECIS element in the 3′-UTR of the mRNA in order to recode the UGA stop codon for Sec insertion. This unique stem–loop structure is the binding site for SBP2 (SECIS-binding protein 2...

View article titled, Regulation and function of selenoproteins in human disease

Includes: Multimedia, Supplementary data

Articles

Modulation of Aβ generation by small ubiquitin-like modifiers does not require conjugation to target proteins

Véronique Dorval, Matthew J. Mazzella, Paul M. Mathews, Ronald T. Hay, Paul E. Fraser

Biochem J (2007) 404 (2): 309–316.

https://doi.org/10.1042/BJ20061451

Published: 14 May 2007

... processing pathway. 1 To whom correspondence should be addressed (email [email protected] ). 21 9 2006 13 2 2007 14 2 2007 14 2 2007 © 2007 Biochemical Society 2007 Alzheimer's disease amyloid-β peptide neurodegeneration sumoylation AD...

View article titled, Modulation of Aβ generation by small ubiquitin-like modifiers does not require conjugation to target proteins

Includes: Supplementary data

Articles

Triosephosphate isomerase deficiency: consequences of an inherited mutation at mRNA, protein and metabolic levels

Judit Oláh, Ferenc Orosz, László G. Puskás, László Hackler, Jr, Margit Horányi, László Polgár, Susan Hollán, Judit Ovádi

Biochem J (2005) 392 (3): 675–683.

https://doi.org/10.1042/BJ20050993

Published: 06 December 2005

...Judit Oláh; Ferenc Orosz; László G. Puskás; László Hackler, Jr; Margit Horányi; László Polgár; Susan Hollán; Judit Ovádi Triosephosphate isomerase (TPI) deficiency is a unique glycolytic enzymopathy coupled with neurodegeneration. Two Hungarian compound heterozygote brothers inherited the same TPI...

View article titled, Triosephosphate isomerase deficiency: consequences of an inherited mutation at mRNA, protein and metabolic levels

Includes: Supplementary data

Articles

More roles for selenoprotein P: local selenium storage and recycling protein in the brain

Des R. RICHARDSON

Biochem J (2005) 386 (2): E5.

https://doi.org/10.1042/BJ20050149

Published: 22 February 2005

... neurodegeneration selenium selenium transport selenoprotein selenoprotein P Selenium (Se) is an essential micronutrient that is required in a variety of metabolic functions that includes its role in antioxidant defences (for a review see [ 1 ]). Se is incorporated into an interesting class of molecules...

View article titled, More roles for selenoprotein P: local selenium storage and recycling protein in the brain

Articles

In brain mitochondria the branched-chain fatty acid phytanic acid impairs energy transduction and sensitizes for permeability transition

Peter SCHÖNFELD, Stefan KAHLERT, Georg REISER

Biochem J (2004) 383 (1): 121–128.

https://doi.org/10.1042/BJ20040583

Published: 24 September 2004

... The Biochemical Society, London 2004 ADP/ATP carrier branched-chain fatty acid mitochondria neurodegeneration permeability transition respiratory chain synaptosome Phytanic acid (3,7,11,15-tetramethylhexadecanoic acid; see structure in Figure 1 ) belongs to the group of 3-methyl-branched...

View article titled, In brain mitochondria the branched-chain fatty acid phytanic acid impairs energy transduction and sensitizes for permeability transition

Articles

Enhanced expression of manganese-dependent superoxide dismutase in human and sheep CLN6 tissues

Claudia HEINE, Jaana TYYNELÄ, Jonathan D. COOPER, David N. PALMER, Milan ELLEDER, Alfried KOHLSCHÜTTER, Thomas BRAULKE

Biochem J (2003) 376 (2): 369–376.

https://doi.org/10.1042/bj20030598

Published: 01 December 2003

[email protected] ). 22 4 2003 28 8 2003 29 8 2003 29 8 2003 The Biochemical Society, London ©2003 2003 fluorescence microscopy lysosomal storage disorder manganese-dependent superoxide dismutase (MnSOD) neurodegeneration neuronal ceroid lipofuscinosis oxidative...

View article titled, Enhanced expression of manganese-dependent superoxide dismutase in human and sheep CLN6 tissues

Articles

Prion peptide induces neuronal cell death through a pathway involving glycogen synthase kinase 3

Mar PÉREZ, Ana I. ROJO, Francisco WANDOSELL, Javier DÍAZ-NIDO, Jesús AVILA

Biochem J (2003) 372 (1): 129–136.

https://doi.org/10.1042/bj20021596

Published: 15 May 2003

... for this neurodegeneration. The PrP 106–126, a synthetic peptide obtained from the amyloidogenic region of the PrP, constitutes a model system to study prion-induced neurodegeneration as it retains the ability to trigger cell death in neuronal cultures. In the present study, we show that the addition of this prion peptide...

View article titled, Prion peptide induces neuronal cell death through a pathway involving glycogen synthase kinase 3

Articles

Metal imbalance and compromised antioxidant function are early changes in prion disease

Alana M. THACKRAY, Robert KNIGHT, Stephen J. HASWELL, Raymond BUJDOSO, David R. BROWN

Biochem J (2002) 362 (2): 253–258.

https://doi.org/10.1042/bj3620253

Published: 22 February 2002

... to the pathological modifications in prion disease. Key words: copper, manganese, neurodegeneration, scrapie, superoxide. METHODS Infection of mice C57BL}6 male and 129Sv female mice were purchased from Harlan UK (Shaw s Farm, Bicester, Oxon., U.K.) and cross- bred in-house to generate F1 wild-type progeny that were...

View article titled, Metal imbalance and compromised antioxidant function are early changes in prion disease

Articles

Nitric oxide inactivation in brain by a novel O2-dependent mechanism resulting in the formation of nitrate ions

Charmaine GRIFFITHS, Bakhtiar YAMINI, Catherine HALL, John GARTHWAITE

Biochem J (2002) 362 (2): 459–464.

https://doi.org/10.1042/bj3620459

Published: 22 February 2002

... was slow (2 h). It is concluded that NO is consumed in the brain through a novel protein, ultimately forming NO $ , and that the slow regeneration of the activity provides a scenario for NO to become toxic. Key words: cGMP, neurodegeneration, respiration. The brain is particularly enriched...

View article titled, Nitric oxide inactivation in brain by a novel O2-dependent mechanism resulting in the formation of nitrate ions

Articles

GSK3 takes centre stage more than 20 years after its discovery

Sheelagh FRAME, Philip COHEN

Biochem J (2001) 359 (1): 1–16.

https://doi.org/10.1042/bj3590001

Published: 24 September 2001

...: cancer, diabetes, insulin, neurodegeneration, Wnt. Akt) [9]. Subsequent work has elucidated the phosphatidyl- inositide (PI) 3-kinase-dependent pathway by which PKB is activated by insulin (Figure 1; for reviews, see [10,11 Thus, in response to insulin, the inhibition of GSK3 promotes...

View article titled, GSK3 takes centre stage more than 20 years after its discovery

Articles

Neuropathy target esterase

Paul GLYNN

Biochem J (1999) 344 (3): 625–631.

https://doi.org/10.1042/bj3440625

Published: 08 December 1999

... residue, and it is suggested that this may cause a toxic gain of function in NTE. The Biochemical Society, London © 1999 1999 active site gain of function neural development neurodegeneration protein family Biochem. J. (1999) 344, 625 631 (Printed in Great Britain) 625 REVIEW ARTICLE...

View article titled, Neuropathy target esterase

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