The response tol-carnitine and glycine t
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C. Sousa; R. A. Chalmers; T. E. Stacey; B. M. Tracey; C. M. Weaver; D. Bradley
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Article
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1986
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Springer
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English
โ 625 KB
The profound metabolic disturbances which occur in isovaleric acidaemia are due to the intramitochondrial accumulation of isovaleryl coenzyme A (CoA) with a consequent reduction in the availability of free CoA. Secondary carnitine insufficiency is also a feature of this and other disorders of organi