Brain energy rescue: an emerging therapeutic concept for neurodegenerative disorders of ageing

SC Cunnane, E Trushina, C Morland… - Nature reviews Drug …, 2020 - nature.com
The brain requires a continuous supply of energy in the form of ATP, most of which is
produced from glucose by oxidative phosphorylation in mitochondria, complemented by …

[HTML][HTML] Role of ROS and nutritional antioxidants in human diseases

Z Liu, Z Ren, J Zhang, CC Chuang… - Frontiers in …, 2018 - frontiersin.org
The overproduction of reactive oxygen species (ROS) has been implicated in the
development of various chronic and degenerative diseases such as cancer, respiratory …

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 …

[HTML][HTML] Mitochondria-targeting drug conjugates for cytotoxic, anti-oxidizing and sensing purposes: current strategies and future perspectives

G Battogtokh, YS Choi, DS Kang, SJ Park… - … Pharmaceutica Sinica B, 2018 - Elsevier
Mitochondrial targeting is a promising approach for solving current issues in clinical
application of chemotherapy and diagnosis of several disorders. Here, we discuss direct …

Spinocerebellar ataxia

T Klockgether, C Mariotti, HL Paulson - Nature reviews Disease primers, 2019 - nature.com
The spinocerebellar ataxias (SCAs) are a genetically heterogeneous group of autosomal
dominantly inherited progressive disorders, the clinical hallmark of which is loss of balance …

Riding the tiger–physiological and pathological effects of superoxide and hydrogen peroxide generated in the mitochondrial matrix

MD Brand - Critical reviews in biochemistry and molecular biology, 2020 - Taylor & Francis
Elevated mitochondrial matrix superoxide and/or hydrogen peroxide concentrations drive a
wide range of physiological responses and pathologies. Concentrations of superoxide and …

[HTML][HTML] TCF4-mediated Fuchs endothelial corneal dystrophy: Insights into a common trinucleotide repeat-associated disease

MP Fautsch, ED Wieben, KH Baratz… - Progress in retinal and …, 2021 - Elsevier
Fuchs endothelial corneal dystrophy (FECD) is a common cause for heritable visual loss in
the elderly. Since the first description of an association between FECD and common …

[HTML][HTML] MitoQ protects dopaminergic neurons in a 6-OHDA induced PD model by enhancing Mfn2-dependent mitochondrial fusion via activation of PGC-1α

Y **, D Feng, K Tao, R Wang, Y Shi, H Qin… - … et Biophysica Acta (BBA …, 2018 - Elsevier
Parkinson's disease (PD) is characterized by the degeneration of dopaminergic neurons in
the substantia nigra compacta (SNc). Although mitochondrial dysfunction is the critical factor …

Neuronal metabolic rewiring promotes resilience to neurodegeneration caused by mitochondrial dysfunction

E Motori, I Atanassov, SMV Kochan… - Science …, 2020 - science.org
Neurodegeneration in mitochondrial disorders is considered irreversible because of limited
metabolic plasticity in neurons, yet the cell-autonomous implications of mitochondrial …

HD and SCA1: Tales from two 30-year journeys since gene discovery

LM Thompson, HT Orr - Neuron, 2023 - cell.com
One of the more transformative findings in human genetics was the discovery that the
expansion of unstable nucleotide repeats underlies a group of inherited neurological …