BACKGROUND: 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. OBJECTIVE: We sought to determine whether the plasma concentration of 3HIA-carnitine increases significantly in marginal biotin deficiency. DESIGN: 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. RESULTS: 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. 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.
BACKGROUND: 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. OBJECTIVE: We sought to determine whether the plasma concentration of 3HIA-carnitine increases significantly in marginal biotin deficiency. DESIGN: 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. RESULTS: 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. 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.
Authors: Shawna L Stratton; Anna Bogusiewicz; Matthew M Mock; Nell I Mock; Amanda M Wells; Donald M Mock Journal: Am J Clin Nutr Date: 2006-08 Impact factor: 7.045
Authors: Thomas D Horvath; Shawna L Stratton; Anna Bogusiewicz; Lindsay Pack; Jeffery Moran; Donald M Mock Journal: Anal Chem Date: 2010-05-15 Impact factor: 6.986
Authors: Katarzyna Urbanska; Evripidis Lanitis; Mathilde Poussin; Rachel C Lynn; Brian P Gavin; Sander Kelderman; Jason Yu; Nathalie Scholler; Daniel J Powell Journal: Cancer Res Date: 2012-02-07 Impact factor: 12.701
Authors: Donald M Mock; Shawna L Stratton; Thomas D Horvath; Anna Bogusiewicz; Nell I Matthews; Cindy L Henrich; Amanda M Dawson; Horace J Spencer; Suzanne N Owen; Gunnar Boysen; Jeffery H Moran Journal: J Nutr Date: 2011-09-14 Impact factor: 4.798
Authors: Shawna L Stratton; Cindy L Henrich; Nell I Matthews; Anna Bogusiewicz; Amanda M Dawson; Thomas D Horvath; Suzanne N Owen; Gunnar Boysen; Jeffery H Moran; Donald M Mock Journal: J Nutr Date: 2011-12-07 Impact factor: 4.798
Authors: Shawna L Stratton; Thomas D Horvath; Anna Bogusiewicz; Nell I Matthews; Cindy L Henrich; Horace J Spencer; Jeffery H Moran; Donald M Mock Journal: J Nutr Date: 2011-01-19 Impact factor: 4.798
Authors: Thomas D Horvath; Nell I Matthews; Shawna L Stratton; Donald M Mock; Gunnar Boysen Journal: Anal Bioanal Chem Date: 2011-09-03 Impact factor: 4.142
Authors: Thomas D Horvath; Shawna L Stratton; Anna Bogusiewicz; Suzanne N Owen; Donald M Mock; Jeffery H Moran Journal: Anal Chem Date: 2010-10-28 Impact factor: 6.986