[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 …
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 …
aging in the heart and other organs, and plays a deleterious role in several age-related …
Peroxisome-mitochondria interplay and disease
Peroxisomes and mitochondria are ubiquitous, highly dynamic organelles with an oxidative
type of metabolism in eukaryotic cells. Over the years, substantial evidence has been …
type of metabolism in eukaryotic cells. Over the years, substantial evidence has been …
Peroxisomes in immune response and inflammation
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 …
organism's overall metabolic status is now recognized as an important and long-overlooked …
X-linked adrenoleukodystrophy: pathogenesis and treatment
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 …
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 …
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 …
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 …
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 …
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 …
brain barrier and acts as a redox cycler. Among its beneficial properties are its abilities to act …