Urinary excretion of 3-hydroxyisovaleryl carnitine is an early and sensitive indicator of marginal biotin deficiency in humans

Journal of Nutrition

Volumn 141, Issue 3, 2011, Pages 353-358

  Stratton, Shawna L ^a   Horvath, Thomas D ^a   Bogusiewicz, Anna ^a   Matthews, Nell I ^a   Henrich, Cindy L ^b   Spencer, Horace J ^a,b   Moran, Jeffery H ^a   Mock, Donald M ^a  

^a UNIVERSITY OF ARKANSAS FOR MEDICAL SCIENCES   (United States)

^b UNIVERSITY OF ARKANSAS FOR MEDICAL SCIENCES   (United States)

Author keywords

[No Author keywords available]

Indexed keywords

3 HYDROXYISOVALERYL CARNITINE; BIOTIN; CARNITINE DERIVATIVE; CREATININE; EGG WHITE; UNCLASSIFIED DRUG;

ADULT; ARTICLE; BIOTIN DEFICIENCY; CREATININE URINE LEVEL; FEMALE; HUMAN; HUMAN EXPERIMENT; LIQUID CHROMATOGRAPHY; NORMAL HUMAN; NUTRITIONAL STATUS; PROTEIN EXPRESSION; TANDEM MASS SPECTROMETRY; URINALYSIS; URINARY EXCRETION; URINE LEVEL; VITAMIN DEFICIENCY; VITAMIN SUPPLEMENTATION;

ADULT; BIOLOGICAL MARKERS; BIOTIN; CARNITINE; EGG WHITE; FEMALE; HUMANS; LYMPHOCYTES; MALE; METHYLMALONYL-COA DECARBOXYLASE; NUTRITIONAL STATUS; REFERENCE VALUES; TIME FACTORS; VITAMIN B DEFICIENCY;

EID: 79951963932     PISSN: 00223166     EISSN: 15416100     Source Type: Journal    
DOI: 10.3945/jn.110.135772     Document Type: Article

References (36)

1. Mock DM, Stadler DD. Conflicting indicators of biotin status from a cross-sectional study of normal pregnancy. J Am Coll Nutr. 1997;16: 252-7. (Pubitemid 27237529)
2. Mock DM, Stadler DD, Stratton SL, Mock NI. Biotin status assessed longitudinally in pregnant women. J Nutr. 1997;127:710-6. (Pubitemid 27200745)
3. Velazquez A, Martin-del-Campo C, Baez A, Zamudio S, Quiterio M, Aguilar JL, Perez-Ortiz B, Sanchez-Ardines M, Guzman-Hernandez J, et al. Biotin deficiency in protein-energy malnutrition. Eur J Clin Nutr. 1998;43:169-73.
4. Krause KH, Berlit P, Bonjour JP. Impaired biotin status in anticonvulsant therapy. Ann Neurol. 1982;12:485-6. (Pubitemid 12006886)
5. Krause KH, Berlit P, Bonjour JP, Schmidt-Gayk H, Schellenberg B, Gillen J. Vitamin status in patients on chronic anticonvulsant therapy. Int J Vitam Nutr Res. 1982;52:375-85. (Pubitemid 13176709)
6. Krause KH, Kochen W, Berlit P, Bonjour JP. Excretion of organic acids associated with biotin deficiency in chronic anticonvulsant therapy. Int J Vitam Nutr Res. 1984;54:217-22. (Pubitemid 14039281)
7. Mock DM, DykenME. Biotin catabolism is accelerated in adults receiving long-term therapy with anticonvulsants. Neurology. 1997;49:1444-7.
8. Mock DM, Mock NI, Lombard KA, Nelson RP. Disturbances in biotin metabolism in children undergoing long-term anticonvulsant therapy. J Pediatr Gastroenterol Nutr. 1998;26:245-50. (Pubitemid 28120024)
9. Said HM. Biotin bioavailability and estimated average requirement: why bother? Am J Clin Nutr. 1999;69:352-3. (Pubitemid 29104552)
10. Roschinger W, Millington DS, Gage DA, Huang ZH, Iwamoto T, Yano S, Packman S, Johnston K, Berry SA, et al. 3-Hydroxyisovalerylcarnitine in patients with deficiency of 3-methylcrotonyl CoA carboxylase. Clin Chim Acta. 1995;240:35-51.
11. Van Hove JL, Josefsberg S, Freehauf C, Thomas JA, Thuy LP, Barshop BA, Woontner M, Mock DM, Chiang PW, et al. Management of a patient with holocarboxylase synthetase deficiency. Mol Genet Metab. 2008;95:201-5.
12. Wolf B. Disorders of biotin metabolism. In: Scriver CR, Beaudet AL, Sly WS, Valle D, editors. The metabolic and molecular basis of inherited disease. 8th ed. New York: McGraw-Hill, Inc; 2001. p. 3151-77.
13. Sweetman L, Williams JC. Branched chain organic acidurias. In: Scriver CR, Beaudet AL, Sly WS, Valle D, editors. The metabolic and molecular basis of inherited disease. 7th ed. New York: McGraw-Hill, Inc; 1995.
14. Zammit VA, Ramsay RR, Bonomini M, Arduini A. Carnitine, mitochondrial function and therapy. Adv Drug Deliv Rev. 2009;61:1353-62.
15. Pande SV. A mitochondrial carnitine acylcarnitine translocase system. Proc Natl Acad Sci USA. 1975;72:883-7.
16. Mock NI, Malik MI, Stumbo PJ, Bishop WP, Mock DM. Increased urinary excretion of 3-hydroxyisovaleric acid and decreased urinary excretion of biotin are sensitive early indicators of decreased status in experimental biotin deficiency. Am J Clin Nutr. 1997;65:951-8. (Pubitemid 27152610)
17. Mock DM, Jackson H, Lankford GL, Mock NI, Weintraub ST. Quantitation of urinary 3-hydroxyisovaleric acid using deuterated 3-hydroxyisovaleric acid as internal standard. Biomed Environ Mass Spectrom. 1989;18:652-6. (Pubitemid 19217782)
18. Stratton SL, Horvath TD, Bogusiewicz A, Matthews NI, Henrich CL, Spencer HJ, Moran JH, Mock DM. Plasma concentration of 3-hydroxyisovaleryl carnitine is an early and sensitive indicator of marginal biotin deficiency in humans. Am J Clin Nutr. 2010;92:1399-405.
19. Horvath TD, Stratton SL, Bogusiewicz A, Owen SO, Mock DM, Moran JH. Quantitative measurement of urinary excretion of 3-hydroxyisovaleryl carnitine by LC-MS/MS as an indicator of biotin status in humans. Anal Chem. 2010;82:9543-8.
20. Mock DM, Henrich CL, Carnell N, Mock NI. Indicators of marginal biotin deficiency and repletion in humans: validation of 3-hydroxyisovaleric acid excretion and a leucine challenge. Am J Clin Nutr. 2002;76:1061-8. (Pubitemid 35244678)
21. NRC. Dietary reference intakes tables: the complete set (PDF). In: Food and Nutrition Board Institute of Medicine, editor. Dietary reference intakes. Washington, DC: The National Academies; 2005.
22. Mock D, Henrich C, Carnell N, Mock N, Swift L. Lymphocyte propionyl-CoA carboxylase and accumulation of odd-chain fatty acid in plasma and erythrocytes are useful indicators of marginal biotin deficiency. J Nutr Biochem. 2002;13:462.
23. Mock DM, Mock NI. Lymphocyte propionyl-CoA carboxylase activity is an early and sensitive indicator of biotin deficiency in rats, but urinary excretion of 3-hydroxyisopropionic acid is not. J Nutr. 2002;132: 21945-50.
24. Sealey WM, Stratton SL, Hansen DK, Mock DM. Marginal maternal biotin deficiency in CD-1 mice reduces fetal mass of biotin-dependent carboxylases. J Nutr. 2005;135:973-7. (Pubitemid 40638293)
25. Stratton SL, Bogusiewicz A, Mock MM, Mock NI, Wells AM, Mock DM. Lymphocyte propionyl-CoA carboxylase and its activation by biotin are sensitive indicators of marginal biotin deficiency in humans. Am J Clin Nutr. 2006;84:384-8. (Pubitemid 44699149)
26. Horvath TD, Stratton SL, Bogusiewicz A, Pack L, Moran JM, Mock DM. Quantitative measurement of plasma 3-hydroxyisovaleryl carnitine by LC-MS/MS as a novel biomarker of biotin status in humans. Anal Chem. 2010;82:4140-4.
27. Mock DM. Rayon balls and disposable-diaper material selectively adsorb creatinine. Am J Clin Nutr. 1992;55:326-30.
28. Mock DM. Marginal biotin deficiency is common in normal human pregnancy and is highly teratogenic in the mouse. J Nutr. 2009;139: 154-7.
29. Stratton SL, Matthews NI, Bogusiewicz A, Zhu S, Mock DM. Evidence that biotin status is impaired in human pregnancy. FASEB J. 2009;23: 103.6.
30. Zempleni J, Mock DM. Marginal biotin deficiency is teratogenic. Proc Soc Exp Biol Med. 2000;223:14-21. (Pubitemid 30182653)
31. Velazquez A, Teran M, Baez A, Gutierrez J, Rodriguez R. Biotin supplementation affects lymphocyte carboxylases and plasma biotin in severe protein-energy malnutrition. Am J Clin Nutr. 1995;61:385-91.
32. Sealey WM, Teague AM, Stratton SL, Mock DM. Smoking accelerates biotin catabolism in women. Am J Clin Nutr. 2004;80:932-5. (Pubitemid 41202093)
33. Strnadova KA, Holub M, Muhl A, Heinze G, Ratschmann R, Mascher H, Stockler-Ipsiroglu S, Waldhauser F, Votava F, et al. Long-term stability of amino acids and acylcarnitines in dried blood spots. Clin Chem. 2007;53:717-22. (Pubitemid 46580243)
34. Shigematsu Y, Bykov IL, Liu YY, Nakai A, Kikawa Y, Sudo M, Fujioka M. Acylcarnitine profile in tissues and body fluids of biotin-deficient rats with and without L-carnitine supplementation. J Inherit Metab Dis. 1994;17:678-90.
35. Pasquali M, Monsen G, Richardson L, AlstonM, Longo N. Biochemical findings in common inborn errors of metabolism. Am J Med Genet C Semin Med Genet. 2006;142C:64-76.
36. Mock DM, Quirk JG, Mock NI. Marginal biotin deficiency during normal pregnancy. Am J Clin Nutr. 2002;75:295-9. (Pubitemid 34101215)