Protective effect of oleic acid against very long-chain fatty acid-induced apoptosis in peroxisome-deficient CHO cells

H Ali, M Yamanishi, K Sunagawa, M Kumon… - … et Biophysica Acta (BBA …, 2024 - Elsevier
Very long-chain fatty acids (VLCFAs) are degraded exclusively in peroxisomes, as
evidenced by the accumulation of VLCFAs in patients with certain peroxisomal disorders …

[HTML][HTML] Pathophysiology of X-Linked Adrenoleukodystrophy: Updates on Molecular Mechanisms

P Parasar, N Kaur, J Singh - Journal of biotechnology and …, 2024 - ncbi.nlm.nih.gov
X-ALD, an inherited monogenic metabolic disorder affecting the CNS and adrenal white
matter, is caused by mutations in ABCD1 gene leading to defective fatty acid oxidation in the …

Imbalanced mitochondrial dynamics contributes to the pathogenesis of X-linked adrenoleukodystrophy

N Launay, J Lopez-Erauskin, P Bianchi, S Guha… - Brain, 2024 - academic.oup.com
The peroxisomal disease adrenoleukodystrophy (X-ALD) is caused by loss of the transporter
of very-long-chain fatty acids (VLCFAs), ABCD1. An excess of VLCFAs disrupts essential …

Fatty Acid Metabolism in Peroxisomes and Related Disorders

K Morito, H Ali, S Kishino, T Tanaka - 2024 - Springer
One of the functions of peroxisomes is the oxidation of fatty acids (FAs). The importance of
this function in our lives is evidenced by the presence of peroxisomal disorders caused by …

Therapie der X-chromosomalen Adrenoleukodystrophie

W Köhler - DGNeurologie, 2024 - Springer
Die X-chromosomale Adrenoleukodystrophie (X-ALD) ist mit einer Inzidenzrate von ca. 1:
15.000 die häufigste Leukodystrophie [1]. Leukodystrophien sind definiert als eine Gruppe …