Journal ArticleDOI
Mutations in the DJ-1 Gene Associated with Autosomal Recessive Early-Onset Parkinsonism
Vincenzo Bonifati,Vincenzo Bonifati,Patrizia Rizzu,Marijke J. van Baren,Onno Schaap,Guido J. Breedveld,Elmar Krieger,Marieke C. J. Dekker,Ferdinando Squitieri,Pablo Ibanez,Marijke Joosse,Jeroen W.F. van Dongen,Nicola Vanacore,Nicola Vanacore,John C. van Swieten,Alexis Brice,Giuseppe Meco,Cornelia M. van Duijn,Ben A. Oostra,Peter Heutink +19 more
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TLDR
It is shown that DJ-1 mutations are associated with PARK7, a monogenic form of human parkinsonism, and these findings indicate that loss ofDJ-1 function leads to neurodegeneration.Abstract:
The DJ-1 gene encodes a ubiquitous, highly conserved protein. Here, we show that DJ-1 mutations are associated with PARK7, a monogenic form of human parkinsonism. The function of the DJ-1 protein remains unknown, but evidence suggests its involvement in the oxidative stress response. Our findings indicate that loss of DJ-1 function leads to neurodegeneration. Elucidating the physiological role of DJ-1 protein may promote understanding of the mechanisms of brain neuronal maintenance and pathogenesis of Parkinson's disease.read more
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Proteomic identification of dopamine-conjugated proteins from isolated rat brain mitochondria and SH-SY5Y cells
TL;DR: A subset of rat brain mitochondrial proteins that were covalently modified by dopamine were observed, including chaperonin, ubiquinol-cytochrome c reductase core protein 1, glucose regulated protein 75/mitochondrial HSP70/mortalin, mitofilin, and mitochondrial creatine kinase.
Journal ArticleDOI
Brain Iron Homeostasis: From Molecular Mechanisms To Clinical Significance and Therapeutic Opportunities
Neena Singh,Swati Haldar,Ajai K. Tripathi,Katharine Horback,Joseph Wong,Deepak Sharma,Amber Beserra,Srinivas Suda,Charumathi Anbalagan,Som Dev,Chinmay K. Mukhopadhyay,Ajay Vir Singh +11 more
TL;DR: Recent information on the regulation of iron uptake and utilization in systemic organs and within the complex environment of the brain is reviewed, with particular emphasis on the underlying mechanisms leading to brain iron mis-metabolism in specific neurodegenerative conditions.
Molecular Mechanisms behind Free RadicalScavengers Function against Oxidative Stress
Fereshteh Ahmadinejad,Simon Geir Møller,Morteza. Hashemzadeh-Chaleshtori,Gholamreza Bidkhori,Mohammad-Saeid Jami +4 more
TL;DR: The cell surviving properties of L-DOPA and Edaravone against oxidative stress conditions rely on the alteration of a number of stress proteins such as Annexin A1, Peroxiredoxin-6 and PARK7/DJ-1 (Parkinson disease protein 7, also known as Protein deglycase DJ-1).
Journal ArticleDOI
shRNA targeting α-synuclein prevents neurodegeneration in a Parkinson’s disease model
Alevtina Zharikov,Alevtina Zharikov,Jason R. Cannon,Jason R. Cannon,Jason R. Cannon,Victor Tapias,Qing Bai,Max Horowitz,Vipul Shah,Amina El Ayadi,Teresa G. Hastings,J. Timothy Greenamyre,J. Timothy Greenamyre,Edward A. Burton,Edward A. Burton +14 more
TL;DR: This paper showed that α-synuclein knockdown is neuroprotective in the rotenone model of sporadic Parkinson's disease and indicate that endogenous SNCA contributes to the specific vulnerability of dopaminergic neurons to systemic mitochondrial inhibition.
Journal ArticleDOI
Best Practices for Generating and Using Alpha-Synuclein Pre-Formed Fibrils to Model Parkinson's Disease in Rodents.
Nicole K. Polinski,Laura A. Volpicelli-Daley,Caryl E. Sortwell,Kelvin C. Luk,Nunilo Cremades,Lindsey M. Gottler,Jessica M. Froula,Megan F. Duffy,Virginia M.-Y. Lee,Terina N. Martinez,Kuldip D. Dave +10 more
TL;DR: Key pitfalls and suggestions for avoiding these mistakes as they relate to generating the aSyn PFFs from monomeric protein, validating the formation of pathogenic aSynPFFs, and using the a synuclein in vivo or in vitro to model PD are identified.
References
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