Metabolism of phytanic acid and 3-methyl-adipic acid excretion in patients with adult Refsum disease
Adult Refsum disease (ARD) is associated with defective α-oxidation of phytanic acid (PA). ω-Oxidation of PA to 3-methyl-adipic acid (3-MAA) occurs although its clinical significance is unclear. In a 40 day study of a new ARD patient, where the plasma half-life of PA was 22.4 days, ω-oxidation accou...
| Main Authors: | , , , , , , , |
|---|---|
| Format: | Article |
| Language: | English |
| Published: |
Elsevier
2003-08-01
|
| Series: | Journal of Lipid Research |
| Subjects: | |
| Online Access: | http://www.sciencedirect.com/science/article/pii/S0022227520337561 |