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Choline and betaine in health and disease

  • Homocysteine and B-Vitamin Metabolism
  • Published:
Journal of Inherited Metabolic Disease

Abstract

Choline is an essential nutrient, but is also formed by de novo synthesis. Choline and its derivatives serve as components of structural lipoproteins, blood and membrane lipids, and as a precursor of the neurotransmitter acetylcholine. Pre-and postnatal choline availability is important for neurodevelopment in rodents. Choline is oxidized to betaine that serves as an osmoregulator and is a substrate in the betaine–homocysteine methyltransferase reaction, which links choline and betaine to the folate-dependent one-carbon metabolism. Choline and betaine are important sources of one-carbon units, in particular, during folate deficiency. Choline or betaine supplementation in humans reduces concentration of total homocysteine (tHcy), and plasma betaine is a strong predictor of plasma tHcy in individuals with low plasma concentration of folate and other B vitamins (B2, B6, and B12) in combination TT genotype of the methylenetetrahydrofolate reductase 677 C->T polymorphism. The link to one-carbon metabolism and the recent availability of food composition data have motivated studies on choline and betaine as risk factors of chronic diseases previously studied in relation to folate and homocysteine status. High intake and plasma level of choline in the mother seems to afford reduced risk of neural tube defects. Intake of choline and betaine shows no consistent relation to cancer or cardiovascular risk or risk factors, whereas an unfavorable cardiovascular risk factor profile was associated with high choline and low betaine concentrations in plasma. Thus, choline and betaine showed opposite relations with key components of metabolic syndrome, suggesting a disruption of mitochondrial choline oxidation to betaine as part of the mitochondrial dysfunction in metabolic syndrome.

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Abbreviations

PC:

Phosphatidylcholine

PE:

Phosphatidylethanolamine

PEMT:

Phosphatidylethanolamine N-methyltransferase

BHMT:

Betaine-homocysteine methyltransferase

tHcy:

Total homocysteine

PML tHcy:

Post-methionine-load tHcy

NAFLD:

Nonalcoholic fatty liver disease

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  1. Section for Pharmacology, Institute of Medicine, University of Bergen, 5021, Bergen, Norway

    Per Magne Ueland

  2. Laboratory of Clinical Biochemistry, Haukeland University Hospital, 5021, Bergen, Norway

    Per Magne Ueland

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Correspondence to Per Magne Ueland.

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Communicated by: Viktor Kozich

Competing interest: None declared.

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Ueland, P.M. Choline and betaine in health and disease. J Inherit Metab Dis 34, 3–15 (2011). https://doi.org/10.1007/s10545-010-9088-4

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