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Hydroxylated long-chain acylcarnitines are biomarkers of mitochondrial myopathy.

08:00 EDT 11th July 2019 | BioPortfolio

Summary of "Hydroxylated long-chain acylcarnitines are biomarkers of mitochondrial myopathy."

Plasma acylcarnitines are biomarkers of beta-oxidation and are useful in diagnosing several inborn errors of metabolism but has never been systematically investigated in patients with mitochondrial myopathy.

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This article was published in the following journal.

Name: The Journal of clinical endocrinology and metabolism
ISSN: 1945-7197
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Medical and Biotech [MESH] Definitions

A mitochondrial protein consisting of four alpha-subunits and four beta-subunits. It contains enoyl-CoA hydratase, long-chain-3-hydroxyacyl-CoA dehydrogenase, and acetyl-CoA C-acyltransferase activities and plays an important role in the metabolism of long chain FATTY ACIDS.

The alpha subunit of mitochondrial trifunctional protein. It contains both enoyl-CoA hydratase activity (EC 4.2.1.17) and long-chain-3-hydroxyacyl-CoA dehydrogenase activity (EC 1.1.1.211). There are four of these alpha subunits in each mitochondrial trifunctional protein molecule.

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A flavoprotein oxidoreductase that has specificity for long-chain fatty acids. It forms a complex with ELECTRON-TRANSFERRING FLAVOPROTEINS and conveys reducing equivalents to UBIQUINONE.

A long-chain fatty acid ester of carnitine which facilitates the transfer of long-chain fatty acids from cytoplasm into mitochondria during the oxidation of fatty acids.

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