Abstract

The long term goal of this research project is to determine the biotin requirement of normal individuals and individuals in specific clinical circumstances. The investigator proposes five specific aims to determine: 1) the usefulness of potential indicators of biotin status. 2) Whether the increased urinary excretion of 3-HIA detected previously in some adults and children treated chronically with anticonvulsant reflects biotin deficiency. 3) Whether increased urinary excretion of 3-HIA detected previously in some pregnant women reflects biotin deficiency. 4) Whether biotin deficiency of similar severity to that observed either early or late in human gestation causes increased rates of fetal malformation in the mouse. 5) The enzyme(s) responsible for biotin beta-oxidation.

  Funding Agency

Agency
National Institute of Health (NIH)
Institute
National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK)
Type
Research Project (R01)
Project #
5R01DK036823-15
Application #
2838085
Study Section
Nutrition Study Section (NTN)
Program Officer
May, Michael K
Project Start
1985-07-01
Project End
2001-11-30
Budget Start
1998-12-05
Budget End
1999-11-30
Support Year
15
Fiscal Year
1999
Total Cost
Indirect Cost

  Institution

Name
Arkansas Children's Hospital Research Institute
Department
Type
DUNS #
City
Little Rock
State
AR
Country
United States
Zip Code
72202

  Publications

Mock, Donald M; Nalbant, Demet; Kyosseva, Svetlana V et al. (2018) Development, validation, and potential applications of biotinylated red blood cells for posttransfusion kinetics and other physiological studies: evidenced-based analysis and recommendations. Transfusion 58:2068-2081
Bogusiewicz, Anna; Horvath, Thomas D; Stratton, Shawna L et al. (2012) Measurement of acylcarnitine substrate to product ratios specific to biotin-dependent carboxylases offers a combination of indicators of biotin status in humans. J Nutr 142:1621-5
Stratton, Shawna L; Horvath, Thomas D; Bogusiewicz, Anna et al. (2011) Urinary excretion of 3-hydroxyisovaleryl carnitine is an early and sensitive indicator of marginal biotin deficiency in humans. J Nutr 141:353-8
Mock, Donald M (2009) Marginal biotin deficiency is common in normal human pregnancy and is highly teratogenic in mice. J Nutr 139:154-7
Van Hove, Johan L K; Josefsberg, Sagi; Freehauf, Cynthia et al. (2008) Management of a patient with holocarboxylase synthetase deficiency. Mol Genet Metab 95:201-5
Mock, Donald M; Bogusiewicz, Anna (2008) Biotin-protein bond: instability and structural modification to provide stability for in vivo applications. Methods Mol Biol 418:209-20
Bogusiewicz, Anna; Mock, Nell I; Mock, Donald M (2005) A biotin-protein bond with stability in plasma. Anal Biochem 337:98-102
Mock, Donald M (2005) Marginal biotin deficiency is teratogenic in mice and perhaps humans: a review of biotin deficiency during human pregnancy and effects of biotin deficiency on gene expression and enzyme activities in mouse dam and fetus. J Nutr Biochem 16:435-7
Bogusiewicz, Anna; Mock, Nell I; Mock, Donald M (2004) Release of biotin from biotinylated proteins occurs enzymatically and nonenzymatically in human plasma. Anal Biochem 331:260-6
Helm, R M; Mock, N I; Simpson, P et al. (2001) Certain immune markers are not good indicators of mild to moderate biotin deficiency in rats. J Nutr 131:3231-6

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