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 …

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 …

Peroxisomal acyl–coenzyme A (acyl‐CoA) oxidase deficiency is an autosomal recessive
inborn error of peroxisomal fatty acid oxidation due to a deficiency of straight‐chain acyl …

Abstract β-oxidation of fatty acids is an important metabolic pathway and is a shared function
between mitochondria and peroxisomes in mammalian cells. On the other hand …

Peroxisomes are dynamic organelles that play an essential role in a variety of metabolic
pathways. Peroxisomal dysfunction can lead to various biochemical abnormalities and result …

Human acyl-CoA oxidase 1 (ACOX1) is a rate-limiting enzyme in peroxisomal fatty acids β-
oxidation and its deficiency is associated with a lethal, autosomal recessive disease, called …

PEX 13 is an integral membrane protein on the peroxisome that regulates peroxisomal
matrix protein import during peroxisome biogenesis. Mutations in PEX 13 and other peroxin …

Very long-chain fatty acids (VLCFAs) are degraded exclusively in peroxisomes, as
evidenced by the accumulation of VLCFAs in patients with certain peroxisomal disorders …

One of the major functions of peroxisomes in mammals is oxidation of very long-chain fatty
acids (VLCFAs). Genetic defects in peroxisomal β-oxidation result in the accumulation of …

Peroxisomal disorders are a clinically and genetically heterogeneous group of diseases
caused by defects in peroxisomal biogenesis or function, usually impairing several …