Plasma concentration of 3-hydroxyisovaleryl carnitine is an early and sensitive indicator of marginal biotin deficiency in humans.

ABSTRACT:

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 bioti

SUBMITTER: Stratton SL 

PROVIDER: S-EPMC2980966 | biostudies-literature | 2010 Dec

REPOSITORIES: biostudies-literature