Evidence of oxidative stress and secondary mitochondrial dysfunction in metabolic and non-metabolic disorders

KM Stepien, R Heaton, S Rankin, A Murphy… - Journal of clinical …, 2017 - mdpi.com
Mitochondrial dysfunction and oxidative stress have been implicated in the pathogenesis of
a number of diseases and conditions. Oxidative stress occurs once the antioxidant defenses …

Brain energy metabolism spurns fatty acids as fuel due to their inherent mitotoxicity and potential capacity to unleash neurodegeneration

P Schönfeld, G Reiser - Neurochemistry international, 2017 - Elsevier
The brain uses long-chain fatty acids (LCFAs) to a negligible extent as fuel for the
mitochondrial energy generation, in contrast to other tissues that also demand high energy …

Tauroursodeoxycholic bile acid arrests axonal degeneration by inhibiting the unfolded protein response in X-linked adrenoleukodystrophy

N Launay, M Ruiz, L Grau, FJ Ortega, EV Ilieva… - Acta …, 2017 - Springer
The activation of the highly conserved unfolded protein response (UPR) is prominent in the
pathogenesis of the most prevalent neurodegenerative disorders, such as Alzheimer's …

Attenuation of 7-ketocholesterol-induced overproduction of reactive oxygen species, apoptosis, and autophagy by dimethyl fumarate on 158 N murine …

A Zarrouk, T Nury, EM Karym, A Vejux… - The Journal of steroid …, 2017 - Elsevier
Mitochondrial dysfunctions and oxidative stress are involved in several non demyelinating or
demyelinating neurodegenerative diseases. Some of them, including multiple sclerosis …

Leukodystrophy: basic and clinical

GV Raymond - Neurodegenerative Diseases: Pathology, Mechanisms …, 2017 - Springer
Leukodystrophies are serious, progressive, genetic disorders of CNS myelin. They may
result from abnormalities of the oligodendrocyte or any of the other of myriad of supporting …

Cytotoxic effects of transthyretin aggregates in an epidermoid cell line

VH Fong, A Vieira - Pathobiology, 2017 - karger.com
Objective: Skin amyloid deposits can occur as part of systemic amyloidoses including those
involving misfolded-aggregated transthyretins (agTTR). Pathological effects of agTTR on the …

In vitro effect of N-acetyl-L-cysteine on glutathione and sulfhydryl levels in X-linked adrenoleukodystrophy patients

DP Marchetti, B Donida, M Deon, CE Jacques… - Clinical and …, 2017 - seer.ufrgs.br
Introduction: Recent evidence shows that oxidative stress seems to be related with the
pathophysiology of X-linked adrenoleukodystrophy (X-ALD), a neurodegenerative disorder …