title: Lymphocyte propionyl-CoA carboxylase and its activation by biotin are sensitive indicators of marginal biotin deficiency in humans.
abstract: BACKGROUND
  Marginal biotin deficiency may be a human teratogen. A biotin status indicator that is not dependent on renal function may be useful in studies of biotin status during pregnancy. A previous study of experimental biotin deficiency suggested that propionyl-coenzyme A carboxylase (PCC) activity in peripheral blood lymphocytes (PBLs) is a sensitive indicator of biotin status.
  
  
  OBJECTIVE
  We examined the utility of measuring PCC activity and the activation of PCC by biotin in detecting marginal biotin deficiency.
  
  
  METHODS
  Marginal biotin deficiency was induced in 7 adults (3 women) by egg-white feeding for 28 d. Blood and urine were obtained on days 0, 14, and 28 (depletion phase) and 44 and 65 (repletion phase). PBLs were incubated with (activated) or without (control) biotin before PCC assay. The activation coefficient of PCC is the ratio of PCC activity in activated PBLs to that in control PBLs. The significance of differences for all measurements was tested by repeated-measures analysis of variance with Fisher's post hoc test and Bonferroni correction.
  
  
  RESULTS
  Changes in the urinary excretion of biotin and of 3-hydroxyisovaleric acid confirmed that marginal biotin deficiency was successfully induced. By day 14, PCC activity had decreased (P < 0.0001) to below the lower limit of normal in all subjects. By day 28, the activation coefficient of PCC had increased significantly (P = 0.003) and was above the upper limit of normal in 6 of 7 subjects.
  
  
  CONCLUSIONS
  PCC activity is the most sensitive indicator of biotin status tested to date. In future pregnancy studies, the use of lymphocyte PCC activity data should prove valuable in the assessment of biotin status.
authors.affiliation: Departments of Biochemistry and Molecular Biology and of Pediatrics, University of Arkansas for Medical Sciences, Little Rock, AR, USA.
authors.forename: Shawna L
authors.initials: SL
authors.lastname: Stratton
authors.forename: Anna
authors.initials: A
authors.lastname: Bogusiewicz
authors.forename: Matthew M
authors.initials: MM
authors.lastname: Mock
authors.forename: Nell I
authors.initials: NI
authors.lastname: Mock
authors.forename: Amanda M
authors.initials: AM
authors.lastname: Wells
authors.forename: Donald M
authors.initials: DM
authors.lastname: Mock
mesh_terms: D000328:Adult; D000704:Analysis of Variance; D015415:Biomarkers / Q000097:blood; D001710:Biotin / Q000097:blood / Q000172:deficiency* / Q000652:urine; D018592:Cross-Over Studies; D003677:Deficiency Diseases / Q000097:blood / Q000175:diagnosis / Q000652:urine; D005260:Female; D006801:Humans; D008214:Lymphocytes / Q000201:enzymology*; D008297:Male; D043586:Methylmalonyl-CoA Decarboxylase / Q000097:blood / Q000378:metabolism*; D015596:Nutrition Assessment; D009752:Nutritional Status; D012680:Sensitivity and Specificity; D014631:Valerates / Q000652:urine
chemical_list: D015415:Biomarkers; D014631:Valerates; C004961:beta-hydroxyisovaleric acid; D001710:Biotin; D043586:Methylmalonyl-CoA Decarboxylase