Biochemistry and genetics of inherited disorders of peroxisomal fatty acid metabolism

Odd Chain Fatty Acids; New Insights of the Relationship Between the Gut Microbiota, Dietary Intake, Biosynthesis and Glucose Intolerance

“…It has been proposed that C17:0 can also be biosynthesised by α -oxidation 18 ; where phytanic acid is the primary target substrate (derived from diet or phytol metabolism 19 ). If C17:0 is biosynthesised through α -oxidation, then any increase in the target substrate; phytanic acid, could change the in vivo C17:0 levels by increasing or inhibiting the rate of C17:0 biosynthesis 20 .…”

Interaction of Phospholipase A/Acyltransferase-3 with Pex19p

“…Peroxisomes are single membrane-surrounded organelles present in virtually all eukaryotic cells and are involved in a wide variety of metabolic events, including scavenging of peroxides and reactive oxygen species, the catabolism of very long chain fatty acids and branched chain fatty acids, and the biosynthesis of all ether-type lipid precursors (1)(2)(3)(4)(5). The physiological importance of peroxisomes has been confirmed by the analysis of a number of inherited diseases exhibiting peroxisome dysfunctions (6,7).…”

“…Metabolic Labeling-Cells were grown at 37°C to 80% confluence in a 6-well plate containing Dulbecco's modified Eagle's medium with 10% fetal calf serum and were labeled with [1][2][3][4][5][6][7][8][9][10][11][12][13][14] C]hexadecanol (1.6 Ci) for 18 h. Cells were then harvested and washed twice with PBS. The total lipids were extracted by the method of Bligh and Dyer (29), spotted on a silica gel thin layer plate (20-cm height), and developed at 4°C for 1 h in hexane/diethyl ether/acetic acid (75:25:1, v/v/v).…”

Lipid metabolism in myelinating glial cells: lessons from human inherited disorders and mouse models

“…The latter may be caused by the failure to degrade arachidonic acidderived eicosanoids ( 72,73 ). Peroxisome biogenesis disorders of Zellweger syndrome occur when any one of 12 peroxins involved in the import of peroxisomal proteins is mutated [for a recent review, see ( 74 )]. The consequent partial or complete peroxisome dysfunction results in defective ␣ -and ␤ -oxidation, subsequent accumulation of fatty acids, leading to myelin defects as also seen in ALD or RD.…”