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1 Introduction 1.1 Relationship among gut microbiota, TMA and TMAO production, and human diseases Consolidated literature from over the last 20 years confirms the relationship between intestinal microbiota and a series of diseases ( 1.2 Biochemical mechanisms of TMA production Most of the TMA is synthesized in the intestine (colon) by the action of some anaerobic commensal gut bacteria containing choline- (or carnitine-) TMA lyase, an enzyme consisting of two units: a catalytic unit (CutC) and its activating enzyme (CutD) ( etc .), betaine, and L-carnitine and its metabolite -butyrobetaine (GBB), releasing TMA after the cleavage of the carbon (C)nitrogen (N) bond of choline/carnitine ( 1.3 Trimethylaminuria (TMAU): etiology, psychosocial impact, and incidence Trimethylaminuria (TMAU) is caused by TMA accumulation