Very long-chain acyl-CoA dehydrogenase (VLCAD)-deficiency is the most common long-chain fatty acid oxidation disorder presenting with heterogeneous phenotypes. Similar to many patients with VLCADD, VLCAD-deficient mice (VLCAD(-/-)) remain asymptomatic over a long period of time. In order to identify the involved compensatory mechanisms, wild-type and VLCAD(-/-) mice were fed one year either wit...

Understanding how functional lipid domains in live cell membranes are generated has posed a challenge. Here, we show that transbilayer interactions are necessary for the generation of cholesterol-dependent nanoclusters of GPI-anchored proteins mediated by membrane-adjacent dynamic actin filaments. We find that long saturated acyl-chains are required for forming GPI-anchor nanoclusters. Simultan...

The synthesis de no2ro of fatty acids by particle-free cell extracts is inhibited by long chain fatty acyl coenzyme A derivatives such as palmityl coenzyme A (l-5). On the other hand, fatty acid synthesis is stimulated by the addition of microsomes (6-9). The enzymes that transfer long chain fatty acyl groups from coenzyme A to glycerol S-phosphate and its derivatives are also located in the mi...

The metabolism of branched-chain amino acids, branched-chain acyl derivatives, d-glucose, l-glutamate, and Mueller-Hinton medium was investigated to determine their effects on the growth, lipid composition, and antibiotic susceptibility of Pseudomonas aeruginosa. The unsaturated fatty acid content of the readily extractable lipids was altered by growth on selected branched-chain amino acids and...

Ceramide (Cer) is involved in the regulation of several cellular processes by mechanisms that depend on Cer-induced changes on membrane biophysical properties. Accumulating evidence shows that Cers with different N-acyl chain composition differentially impact cell physiology, which may in part be due to specific alterations in membrane biophysical properties. We now address how the sphingolipid...

In recent years long chain fatty acids have been shown to be the principal metabolic fuel of the adult heart (1-3). For the tissues of the mammalian fetus, however, carbohydrates rather than lipids appear to serve as the primary source of energy (4, 5). This difference in energy metabolism related to maturation suggested that the heart of the newborn, in contrast to that of the adult, might be ...

We recently reported a new metabolic competency for Escherichia coli, the ability to degrade and utilize fatty acids of various chain lengths as sole carbon and energy sources. This beta-oxidation pathway is distinct from the previously described aerobic fatty acid degradation pathway and requires enzymes encoded by two operons, yfcYX and ydiQRSTD. The yfcYX operon (renamed fadIJ) encodes enzym...

Acyl lipids are essential constituents of all cells, but acyl chain requirements vary greatly and depend on the cell type considered. This implies a tight regulation of fatty acid production so that supply fits demand. Isolation of the Arabidopsis thaliana WRINKLED1 (WRI1) transcription factor established the importance of transcriptional regulation for modulating the rate of acyl chain product...