[HTML][HTML] X-linked adrenoleukodystrophy: clinical, metabolic, genetic and pathophysiological aspects

S Kemp, J Berger, P Aubourg - … Et Biophysica Acta (BBA)-Molecular Basis …, 2012 - Elsevier
X-linked adrenoleukodystrophy (X-ALD) is the most frequent peroxisomal disease. The two
main clinical phenotypes of X-ALD are adrenomyeloneuropathy (AMN) and inflammatory …

Mitochondrial ROS and mitochondria-targeted antioxidants in the aged heart

D Bou-Teen, N Kaludercic, D Weissman… - Free Radical Biology …, 2021 - Elsevier
Excessive mitochondrial ROS production has been causally linked to the pathophysiology of
aging in the heart and other organs, and plays a deleterious role in several age-related …

Peroxisome-mitochondria interplay and disease

M Schrader, J Costello, LF Godinho… - Journal of inherited …, 2015 - Springer
Peroxisomes and mitochondria are ubiquitous, highly dynamic organelles with an oxidative
type of metabolism in eukaryotic cells. Over the years, substantial evidence has been …

Peroxisomes in immune response and inflammation

F Di Cara, P Andreoletti, D Trompier, A Vejux… - International journal of …, 2019 - mdpi.com
The immune response is essential to protect organisms from infection and an altered self. An
organism's overall metabolic status is now recognized as an important and long-overlooked …

X-linked adrenoleukodystrophy: pathogenesis and treatment

M Engelen, S Kemp, BT Poll-The - Current neurology and neuroscience …, 2014 - Springer
Abstract X-linked adrenoleukodystrophy (X-ALD) is a puzzling inborn error of metabolism
with a strikingly heterogeneous clinical spectrum. All patients have mutations in the ABCD1 …

[HTML][HTML] Peroxisomal ABC transporters: structure, function and role in disease

M Morita, T Imanaka - Biochimica et Biophysica Acta (BBA)-Molecular Basis …, 2012 - Elsevier
ATP-binding cassette (ABC) transporters belong to one of the largest families of membrane
proteins, and are present in almost all living organisms from eubacteria to mammals. They …

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 …

The peroxisome: an update on mysteries

M Islinger, S Grille, HD Fahimi, M Schrader - Histochemistry and cell …, 2012 - Springer
Peroxisomes contribute to several crucial metabolic processes such as β-oxidation of fatty
acids, biosynthesis of ether phospholipids and metabolism of reactive oxygen species …

How the brain fights fatty acids' toxicity

P Schönfeld, G Reiser - Neurochemistry International, 2021 - Elsevier
Neurons spurn hydrogen-rich fatty acids for energizing oxidative ATP synthesis, contrary to
other cells. This feature has been mainly attributed to a lower yield of ATP per reduced …

Methylene blue upregulates Nrf2/ARE genes and prevents tau-related neurotoxicity

C Stack, S Jainuddin, C Elipenahli… - Human molecular …, 2014 - academic.oup.com
Methylene blue (MB, methylthioninium chloride) is a phenothiazine that crosses the blood
brain barrier and acts as a redox cycler. Among its beneficial properties are its abilities to act …