Adrenoleukodystrophy (ALD) is a rare X‐linked disease caused by a mutation of the
peroxisomal ABCD1 gene. This review summarizes our current understanding of the …

Peroxisomes are key metabolic organelles, which contribute to cellular lipid metabolism, eg
the β-oxidation of fatty acids and the synthesis of myelin sheath lipids, as well as cellular …

Background & Aims Severe malnutrition in young children is associated with signs of hepatic
dysfunction such as steatosis and hypoalbuminemia, but its etiology is unknown …

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 contain numerous enzymatic activities that are important for mammalian
physiology. Patients lacking either all peroxisomal functions or a single enzyme or …

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 …

Beyond the disorders recognized as mitochondrial diseases, abnormalities in function
and/or ultrastructure of mitochondria have been reported in several unrelated pathologies …

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 …

X-linked adrenoleukodystrophy (X-ALD), a potentially fatal neurometabolic disorder with no
effective pharmacological treatment, is characterized by clinical manifestations ranging from …

N-acetylcysteine (NAC), a precursor of cysteine and, thereby, glutathione (GSH), acts as an
antioxidant through a variety of mechanisms, including oxidant scavenging, GSH …