▪ Abstract The synthesis and excretion of bile acids comprise the major pathway of
cholesterol catabolism in mammals. Synthesis provides a direct means of converting …

In humans, peroxisomes harbor a complex set of enzymes acting on various lipophilic
carboxylic acids, organized in two basic pathways, α-oxidation and β-oxidation; the latter …

Protein lysine acetylation has emerged as a key posttranslational modification in cellular
regulation, in particular through the modification of histones and nuclear transcription …

Almost a century has passed since Knoop performed his classical feeding experiments that
led him to propose the principle of fl-oxidation for the degradation of fatty acids. After …

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

Peroxisomal disorders are a group of inherited diseases in man in which either peroxisome
biogenesis or one or more peroxisomal functions are impaired. The peroxisomal disorders …

The triple A syndrome (MIM 231550) is a rare autosomal recessive disorder characterized
by adrenal insufficiency, achalasia and alacrima. The frequent association with a variety of …

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Peroxisomes catalyse a large variety of different cellular functions of which most have to do
with lipid metabolism. This paper deals with the role of peroxisomes in three key pathways of …

Fatty acids with greater than 22 carbon atoms (very long chain fatty acids, VLCFA) are
present in small amounts in most animal tissues. Saturated and monoenoic VLCFA are …