Deficiency of very long-chain acyl-CoA dehydrogenase (VLCAD) results in accumulation of C14-C18 acylcarnitines and low free carnitine. Carnitine supplementation is still controversial. VLCAD knockout (VLCAD(+/-)) mice exhibit a similar clinical and biochemical phenotype to those observed in humans. VLCAD(+/-) mice were fed with carnitine dissolved in drinking water. Carnitine, acylcarnitines, and gamma-butyrobetaine were measured in blood and tissues. Measurements were performed under resting conditions, after exercise and after 24 h of regeneration. HepG2 cells were incubated with palmitoyl-CoA and palmitoyl-carnitine, respectively, to examine toxicity. With carnitine supplementation, acylcarnitine production was significantly induced. Nevertheless, carnitine was low in skeletal muscle after exercise. Without carnitine supplementation, liver carnitine significantly increased after exercise, and after 24 h of regeneration, carnitine concentrations in skeletal muscle completely replenished to initial values. Incubation of hepatic cells with palmitoyl-CoA and palmitoyl-carnitine revealed a significantly reduced cell viability after incubation with palmitoyl-carnitine. The present study demonstrates that carnitine supplementation results in significant accumulation of potentially toxic acylcarnitines in tissues. The expected prevention of low tissue carnitine was not confirmed. The principle mechanism regulating carnitine homeostasis seems to be endogenous carnitine biosynthesis, also under conditions with increased demand of carnitine such as in VLCAD-deficiency.
We characterize the modules and the complexes involved in the
Dualities induced by a 1-cotilting bimodule in terms of a linear compactness
Condition. Our result generalizes the classical characterization of reflexive
Modules with respect to Morita dualities. The linear compactness notion
We propose that all these
Observations can naturally be understood in terms of the forward scattering
Impurities located off the CuO2 planes. This is substantiated by combining the
Eliashberg calculations including the forward scattering impurities and
Analysis of laser based ARPES intensity from Bi...
We have demonstrated the occurrence of multi-stable
States in a system of phase oscillators that are dynamically coupled. We find
That the multi-stable state is comprised of a two cluster synchronization state
Where the clusters are in anti-phase relationship with each other and a
Pubget Updates sends you emails when Pubget finds new papers that match your search. Use Pubget Updates to get the latest articles for your specialty, written by a colleague, or published by your favorite journals.