Fatty acid omega‐oxidation as a rescue pathway for fatty acid oxidation disorders in humans

RJA Wanders, J Komen, S Kemp - The FEBS journal, 2011 - Wiley Online Library
RJA Wanders, J Komen, S Kemp
The FEBS journal, 2011Wiley Online Library
Fatty acids (FAs) can be degraded via different mechanisms including α‐, β‐and ω‐
oxidation. In humans, a range of different genetic diseases has been identified in which
either mitochondrial FA β‐oxidation, peroxisomal FA β‐oxidation or FA α‐oxidation is
impaired. Treatment options for most of these disorders are limited. This has prompted us to
study FA ω‐oxidation as a rescue pathway for these disorders, based on the notion that if the
ω‐oxidation of specific FAs could be upregulated one could reduce the accumulation of …
Fatty acids (FAs) can be degraded via different mechanisms including α‐, β‐ and ω‐oxidation. In humans, a range of different genetic diseases has been identified in which either mitochondrial FA β‐oxidation, peroxisomal FA β‐oxidation or FA α‐oxidation is impaired. Treatment options for most of these disorders are limited. This has prompted us to study FA ω‐oxidation as a rescue pathway for these disorders, based on the notion that if the ω‐oxidation of specific FAs could be upregulated one could reduce the accumulation of these FAs and the subsequent detrimental effects in the different groups of disorders. In this minireview, we describe our current state of knowledge in this area with special emphasis on Refsum disease and X‐linked adrenoleukodystrophy.
Wiley Online Library