In HepG2 cells, coexisting carnitine deficiency masks important indicators of marginal biotin deficiency.

ABSTRACT:

Background

A large number of birth defects are related to nutrient deficiencies; concern that biotin deficiency is teratogenic in humans is reasonable. Surprisingly, studies indicate that increased urinary 3-hydroxyisovalerylcarnitine (3HIAc), a previously validated marker of biotin deficiency, is not a valid biomarker in pregnancy.

Objective

In this study we hypothesized that coexisting carnitine deficiency can prevent the increase in 3HIAc due to biotin deficiency.

Methods

We used a 2-factor nutrient depletion design to induce isolated and combined biotin and carnitine deficiency in HepG2 cells and then repleted cells with carnitine. To elucidate the metabolic pathogenesis, we quantitated intracellular and extracellular free carnitine, acylcarnitines, and acylcarnit

SUBMITTER: Bogusiewicz A 

PROVIDER: S-EPMC4264022 | biostudies-literature | 2015 Jan

REPOSITORIES: biostudies-literature