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Improvement in exercise tolerance in isovaleric acidaemia with L-carnitine therapy

โœ Scribed by P.J. Lee; E.L. Harrison; M.G. Jones; R.A. Chalmers; J.V. Leonard; B.J. Whipp

Book ID
110224352
Publisher
Springer
Year
1998
Tongue
English
Weight
85 KB
Volume
21
Category
Article
ISSN
0141-8955

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โœ C. Sousa; R. A. Chalmers; T. E. Stacey; B. M. Tracey; C. M. Weaver; D. Bradley ๐Ÿ“‚ Article ๐Ÿ“… 1986 ๐Ÿ› Springer ๐ŸŒ English โš– 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