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Respir Physiol 2000, 122: 209221

Am J Med Genet A

Upon proteolytic degradation, these residues undergo a series of enzymatic reactionsincluding hydroxylation, aldol cleavage, and aldehyde oxidationleading to the formation of 4-trimethylaminobutyric acid (-butyrobetaine), which is subsequently converted into l-carnitine through the catalytic action of -butyrobetaine dioxygenase (BBOX1) 25,26

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