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. 2021 Mar 13;10(6):1202. doi: 10.3390/jcm10061202

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© 2021 by the authors.

Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (http://creativecommons.org/licenses/by/4.0/).

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Carnitine biosynthetic pathway. Abbreviation: enzymes: 1, S-adenosyl methionine: ε-N-lysine methyltransferase; 2, protease (lysosomes); 3, ε-N-trimethyllysine dioxygenase (TMLD*) (mitochondria); 4, β-hydroxy-ε-N-trimethyllysine aldolase (HTMLA) (cytosol); 5, 4-N-trimethylaminobutyraldehyde dehydrogenase (TMABA-DH) (cytosol); 6, γ-butyrobetaine dioxygenase (BBD*) (cytosol); second substrates and products: (a) S-adenosyl methionine (SAM)→ S-adenosyl homocysteine (SAH); (b) 2-ketoglutarate + 02 →succinate + C02; (d) NAD+ → NADH + H+; (e) 2-ketoglutarate + 02 → succinate + C02; coenzymes: (b) vitamin C, iron; (c) pyridoxal phosphate; (e) vitamin C, iron. * genetically determined enzyme deficiency.

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