| First Author | Wanders RJ | Year | 2003 |
| Journal | Biochim Biophys Acta | Volume | 1631 |
| Issue | 2 | Pages | 119-35 |
| PubMed ID | 12633678 | Mgi Jnum | J:82730 |
| Mgi Id | MGI:2654967 | Doi | 10.1016/s1388-1981(03)00003-9 |
| Citation | Wanders RJ, et al. (2003) Phytanic acid alpha-oxidation, new insights into an old problem: a review. Biochim Biophys Acta 1631(2):119-35 |
| abstractText | Phytanic acid (3,7,10,14-tetramethylhexadecanoic acid) is a branched-chain fatty acid which is known to accumulate in a number of different genetic diseases including Refsum disease. Due to the presence of a methyl-group at the 3-position, phytanic acid and other 3-methyl fatty acids can not undergo beta-oxidation but are first subjected to fatty acid alpha-oxidation in which the terminal carboxyl-group is released as CO(2). The mechanism of alpha-oxidation has long remained obscure but has been resolved in recent years. Furthermore, peroxisomes have been found to play an indispensable role in fatty acid alpha-oxidation, and the complete alpha-oxidation machinery is probably localized in peroxisomes. This Review describes the current state of knowledge about fatty acid alpha-oxidation in mammals with particular emphasis on the mechanism involved and the enzymology of the pathway. |
