Concepedia

Hepatic and Muscular Presentations of Carnitine Palmitoyl Transferase Deficiency: Two Distinct Entities

France Demaugre, Jean‐Paul Bonnefont, Grant Mitchell, Anna Pelet, Marco Rimoldi, Stefano Di Donato, Jean-Marie Saudubray

Pediatric Research · 1988 · 131 citations · 9 references

DOI

Concepts

References

9

Carnitine palmitoyltransferase I. The site of inhibition of hepatic fatty acid oxidation by malonyl-CoA.

Julie McGarry, G.F. Leatherman, Daniel W. Foster · Journal of Biological Chemistry · 1978 · 602 citations · Full text

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Fasting hypoglycemia resulting from hepatic carnitine palmitoyl transferase deficiency

P.F. Bougneres, Jean-M. Saudubray, C. Marsac et al. · The Journal of Pediatrics · 1981 · 179 citations

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Carnitine Metabolism and Deficiency Syndromes

Charles J. Rebouche, Andrew G. Engel · Mayo Clinic Proceedings · 1983 · 156 citations

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Regulatory properties of a mutant carnitine palmitoyltransferase in human skeletal muscle

Stephan Zierz, Andrew G. Engel · European Journal of Biochemistry · 1985 · 89 citations · Full text

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Mechanism of carnitine acylcarnitine translocase-catalyzed import of acylcarnitines into mitochondria.

M S Murthy, S.V. Pande · Journal of Biological Chemistry · 1984 · 87 citations · Full text

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