Literature DB >> 12399279

Indicators of marginal biotin deficiency and repletion in humans: validation of 3-hydroxyisovaleric acid excretion and a leucine challenge.

Donald M Mock1, Cindy L Henrich, Nadine Carnell, Nell I Mock.   

Abstract

BACKGROUND: The results of clinical studies have provided evidence that marginal biotin deficiency is more common than was previously thought. A previous study of 10 subjects showed that the urinary excretion of biotin and 3-hydroxyisovaleric acid (3HIA) are early and sensitive indicators of marginal biotin deficiency.
OBJECTIVE: Marginal biotin deficiency was experimentally induced and corrected to assess the utility of 3 indicators of biotin status: urinary excretion of biotin and 3HIA and the increase in 3HIA excretion after leucine loading.
DESIGN: Eleven healthy adults consumed an egg white diet for 28 d. Blood and 24-h urine samples were collected before the start of the diet and twice weekly thereafter. In 5 subjects, an oral leucine challenge was performed weekly for 4 wk. After depletion, biotin status was restored with a general diet with or without a supplement containing 80 micro g biotin. Urinary excretion of biotin, bisnorbiotin, and biotin sulfoxides was determined by avidin-binding assay after HPLC. Excretion of 3HIA, an indicator of reduced activity of the biotin-dependent enzyme methylcrotonyl-CoA carboxylase (EC 6.4.1.4), was measured by gas chromatography-mass spectrometry.
RESULTS: 3HIA excretion increased significantly with time on the egg white diet (P < 0.0001), as did 3HIA excretion in response to the leucine challenge (P < 0.002); the excretion of both biotin and bisnorbiotin decreased significantly with time (P < 0.0001). In most subjects, biotin status returned to normal after 1 wk of a general diet.
CONCLUSIONS: Excretion of 3HIA and of biotin are early and sensitive indicators of biotin deficiency. 3HIA excretion after a leucine challenge is at least as sensitive.

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Year:  2002        PMID: 12399279      PMCID: PMC1435357          DOI: 10.1093/ajcn/76.5.1061

Source DB:  PubMed          Journal:  Am J Clin Nutr        ISSN: 0002-9165            Impact factor:   7.045


  26 in total

Review 1.  Marginal biotin deficiency is teratogenic.

Authors:  J Zempleni; D M Mock
Journal:  Proc Soc Exp Biol Med       Date:  2000-01

2.  Serum concentrations of bisnorbiotin and biotin sulfoxide increase during both acute and chronic biotin supplementation.

Authors:  D M Mock; N I Mock
Journal:  J Lab Clin Med       Date:  1997-03

3.  Increased urinary excretion of 3-hydroxyisovaleric acid and decreased urinary excretion of biotin are sensitive early indicators of decreased biotin status in experimental biotin deficiency.

Authors:  N I Mock; M I Malik; P J Stumbo; W P Bishop; D M Mock
Journal:  Am J Clin Nutr       Date:  1997-04       Impact factor: 7.045

4.  Conflicting indicators of biotin status from a cross-sectional study of normal pregnancy.

Authors:  D M Mock; D D Stadler
Journal:  J Am Coll Nutr       Date:  1997-06       Impact factor: 3.169

5.  Purification and crystallization of avidin.

Authors:  N M Green; E J Toms
Journal:  Biochem J       Date:  1970-06       Impact factor: 3.857

6.  Biotin analysis of commercial vitamin and other nutritional supplements.

Authors:  N Tolaymat; D M Mock
Journal:  J Nutr       Date:  1989-10       Impact factor: 4.798

7.  Biotin biochemistry and human requirements.

Authors:  J Zempleni; D M Mock
Journal:  J Nutr Biochem       Date:  1999-03       Impact factor: 6.048

8.  Vitamin status in patients on chronic anticonvulsant therapy.

Authors:  K H Krause; P Berlit; J P Bonjour; H Schmidt-Gayk; B Schellenberg; J Gillen
Journal:  Int J Vitam Nutr Res       Date:  1982       Impact factor: 1.784

9.  Excretion of organic acids associated with biotin deficiency in chronic anticonvulsant therapy.

Authors:  K H Krause; W Kochen; P Berlit; J P Bonjour
Journal:  Int J Vitam Nutr Res       Date:  1984       Impact factor: 1.784

10.  Biotin accounts for only half of the total avidin-binding substances in human serum.

Authors:  D M Mock; G L Lankford; N I Mock
Journal:  J Nutr       Date:  1995-04       Impact factor: 4.798

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  26 in total

Review 1.  Novel roles of holocarboxylase synthetase in gene regulation and intermediary metabolism.

Authors:  Janos Zempleni; Dandan Liu; Daniel Teixeira Camara; Elizabeth L Cordonier
Journal:  Nutr Rev       Date:  2014-03-28       Impact factor: 7.110

2.  Increasing longevity by tuning up metabolism. To maximize human health and lifespan, scientists must abandon outdated models of micronutrients.

Authors:  Bruce N Ames
Journal:  EMBO Rep       Date:  2005-07       Impact factor: 8.807

3.  Biotin requirements are lower in human Jurkat lymphoid cells but homeostatic mechanisms are similar to those of HepG2 liver cells.

Authors:  Gaganpreet Kaur Mall; Yap Ching Chew; Janos Zempleni
Journal:  J Nutr       Date:  2010-03-31       Impact factor: 4.798

Review 4.  Biotin: From Nutrition to Therapeutics.

Authors:  Donald M Mock
Journal:  J Nutr       Date:  2017-07-12       Impact factor: 4.798

5.  Lymphocyte propionyl-CoA carboxylase and its activation by biotin are sensitive indicators of marginal biotin deficiency in humans.

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

6.  Development and characterization of a mouse with profound biotinidase deficiency: a biotin-responsive neurocutaneous disorder.

Authors:  Kirit Pindolia; Megan Jordan; Caiying Guo; Nell Matthews; Donald M Mock; Erin Strovel; Miriam Blitzer; Barry Wolf
Journal:  Mol Genet Metab       Date:  2010-10-13       Impact factor: 4.797

7.  Urinary excretion of 3-hydroxyisovaleric acid and 3-hydroxyisovaleryl carnitine increases in response to a leucine challenge in marginally biotin-deficient humans.

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

8.  Smoking accelerates biotin catabolism in women.

Authors:  Wendy M Sealey; April M Teague; Shawna L Stratton; Donald M Mock
Journal:  Am J Clin Nutr       Date:  2004-10       Impact factor: 7.045

9.  Marginal biotin deficiency is common in normal human pregnancy and is highly teratogenic in mice.

Authors:  Donald M Mock
Journal:  J Nutr       Date:  2008-12-03       Impact factor: 4.798

10.  Marginal biotin deficiency is teratogenic in ICR mice.

Authors:  Donald M Mock; Nell I Mock; Christopher W Stewart; James B LaBorde; Deborah K Hansen
Journal:  J Nutr       Date:  2003-08       Impact factor: 4.798

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