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Abstract
Blood-based indicators of biotin status in humans were shown to be useful tools in several clinical situations, including pregnancy. We previously validated the activity of the biotin-dependent enzyme propionyl-coenzyme A carboxylase (PCC) in lymphocytes as a sensitive and specific blood-based indicator of marginal degrees of biotin deficiency. However, the measurement of PCC activity in population studies presents substantial analytic challenges. 3-Hydroxyisovaleryl carnitine (3HIA-carnitine) increases in response to the decreased activity of the biotin-dependent enzyme methylcrotonyl-coenzyme A carboxylase and might reflect biotin status. We sought to determine whether the plasma concentration of 3HIA-carnitine increases significantly in marginal biotin deficiency. We experimentally induced marginal, asymptomatic biotin deficiency in 10 healthy adults (8 women) by having the subjects consume undenatured egg white for 28 d; biotin status was then repleted. Plasma concentrations of 3HIA-carnitine were measured on days 0, 14, 28, 35, and 50 by liquid chromatography-mass spectroscopy. The mean plasma 3HIA-carnitine concentration increased with depletion (P < 0.0001) and decreased with repletion (P < 0.0001). Plasma 3HIA-carnitine concentrations were greater than the upper limit of normal concentrations in 7 of 10 subjects by day 14 and in 9 of 10 subjects by day 28 and decreased to within normal limits in 9 of 10 subjects by day 50. These studies provide evidence that 3HIA-carnitine is an early and sensitive indicator of marginal biotin deficiency. The ease of sample collection, small sample volume requirement, and stability of 3HIA-carnitine during storage suggest that plasma 3HIA-carnitine concentration is likely to be a useful indicator of marginal biotin deficiency for larger population studies.
Highlights
Biotin is a water-soluble vitamin that is generally classified in the B group
We report an assessment of the validity of the plasma concentration of 3HIA-carnitine as an indicator of marginal biotin deficiency measured by using a liquid chromatography– tandem mass spectroscopy (LC-MS/MS) method that was recently developed by our laboratory [18]
Marginal biotin deficiency was successfully induced in the depletion phase of the study by the diet of low biotin plus egg white as judged by decreased propionyl-coenzyme A carboxylase (PCC) activities (Figure 1A) and increased urinary 3-hydroxyisovaleric acid (3-HIA) excretions (Figure 1B)
Summary
Biotin is a water-soluble vitamin that is generally classified in the B group. Mounting evidence indicates that marginal biotin deficiency is not rare, which is contrary to previous assumptions. Early and sensitive indicators of biotin status are likely to be useful [9]. We previously validated the activity of the biotindependent enzyme propionyl-coenzyme A carboxylase (PCC) in lymphocytes as a sensitive and specific blood-based indicator of marginal degrees of biotin deficiency. 3-Hydroxyisovaleryl carnitine (3HIA-carnitine) increases in response to the decreased activity of the biotin-dependent enzyme methylcrotonyl-coenzyme A carboxylase and might reflect biotin status. Objective: We sought to determine whether the plasma concentration of 3HIA-carnitine increases significantly in marginal biotin deficiency. Conclusions: These studies provide evidence that 3HIA-carnitine is an early and sensitive indicator of marginal biotin deficiency. The ease of sample collection, small sample volume requirement, and stability of 3HIA-carnitine during storage suggest that plasma 3HIA-carnitine concentration is likely to be a useful indicator of marginal biotin deficiency for larger population studies.
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