Regulatory properties of a mutant carnitine palmitoyltransferase in human skeletal muscle.

@article{Zierz1985RegulatoryPO,
  title={Regulatory properties of a mutant carnitine palmitoyltransferase in human skeletal muscle.},
  author={Stephan Zierz and Andrew George Engel},
  journal={European journal of biochemistry},
  year={1985},
  volume={149 1},
  pages={
          207-14
        }
}
Carnitine palmitoyltransferase (EC 2.3.1.21) was studied in sonicated muscle homogenates of seven patients who had recurrent attacks of myoglobinuria and marked deficiency of carnitine palmitoyltransferase in the isotope exchange assay, and in control subjects. When L-palmitoylcarnitine was reduced from 0.5 mM to 0.05 mM in the isotope exchange assay, enzyme activity returned to normal in the patients but was not significantly altered in the controls. When the forward assay was performed in the… CONTINUE READING
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