Oxidative stress in neurodegenerative diseases: from molecular mechanisms to clinical applications

Z Liu, T Zhou, AC Ziegler, P Dimitrion… - Oxidative medicine and …, 2017 - Wiley Online Library
Increasing numbers of individuals, particularly the elderly, suffer from neurodegenerative
disorders. These diseases are normally characterized by progressive loss of neuron cells …

Mitochondrial dysfunction in the development and progression of neurodegenerative diseases

J Johnson, E Mercado-Ayon, Y Mercado-Ayon… - Archives of biochemistry …, 2021 - Elsevier
In addition to ATP synthesis, mitochondria are highly dynamic organelles that modulate
apoptosis, ferroptosis, and inflammasome activation. Through executing these varied …

[HTML][HTML] Cellular iron uptake, trafficking and metabolism: Key molecules and mechanisms and their roles in disease

DJR Lane, AM Merlot, MLH Huang, DH Bae… - … et Biophysica Acta (BBA …, 2015 - Elsevier
Iron is a crucial transition metal for virtually all life. Two major destinations of iron within
mammalian cells are the cytosolic iron-storage protein, ferritin, and mitochondria. In …

Trinucleotide repeat disorders

HT Orr, HY Zoghbi - Annu. Rev. Neurosci., 2007 - annualreviews.org
The discovery that expansion of unstable repeats can cause a variety of neurological
disorders has changed the landscape of disease-oriented research for several forms of …

Variable tandem repeats accelerate evolution of coding and regulatory sequences

R Gemayel, MD Vinces, M Legendre… - Annual review of …, 2010 - annualreviews.org
Genotype-to-phenotype mapping commonly focuses on two major classes of mutations:
single nucleotide polymorphisms (SNPs) and copy number variation (CNV). Here, we …

Mitochondrial iron trafficking and the integration of iron metabolism between the mitochondrion and cytosol

DR Richardson, DJR Lane… - Proceedings of the …, 2010 - National Acad Sciences
The mitochondrion is well known for its key role in energy transduction. However, it is less
well appreciated that it is also a focal point of iron metabolism. Iron is needed not only for …

Diseases of unstable repeat expansion: mechanisms and common principles

JR Gatchel, HY Zoghbi - Nature Reviews Genetics, 2005 - nature.com
The list of developmental and degenerative diseases that are caused by expansion of
unstable repeats continues to grow, and is now approaching 20 disorders. The pathogenic …

Oxidative stress and the homeodynamics of iron metabolism

N Bresgen, PM Eckl - Biomolecules, 2015 - mdpi.com
Iron and oxygen share a delicate partnership since both are indispensable for survival, but if
the partnership becomes inadequate, this may rapidly terminate life. Virtually all cell …

Iron behaving badly: inappropriate iron chelation as a major contributor to the aetiology of vascular and other progressive inflammatory and degenerative diseases

DB Kell - BMC medical genomics, 2009 - Springer
Background The production of peroxide and superoxide is an inevitable consequence of
aerobic metabolism, and while these particular'reactive oxygen species'(ROSs) can exhibit a …

Mouse models for Friedreich ataxia exhibit cardiomyopathy, sensory nerve defect and Fe-S enzyme deficiency followed by intramitochondrial iron deposits

H Puccio, D Simon, M Cossée, P Criqui-Filipe… - Nature …, 2001 - nature.com
Friedreich ataxia (FRDA), the most common autosomal recessive ataxia, is characterized by
degeneration of the large sensory neurons and spinocerebellar tracts, cardiomyopathy and …