beta-hydroxyisovaleric acid

Eley HL, Russell ST, Baxter JH, Mukerji P, Tisdale MJ. Signaling pathways initiated by -hydroxy-methylbutyrate to attenuate the… (More)

Summary: Since the addition of tandem mass spectrometry (MS/MS) to the North Carolina Newborn Screening Program, 20 infants with… (More)

The incidence of marginal biotin deficiency in normal human gestation is approximately one in three. In ICR mice, maternal biotin… (More)

3-Methylglutaconic aciduria type I is an autosomal recessive disorder clinically characterized by various symptoms ranging from… (More)

BACKGROUND Biotin deficiency is teratogenic in several mammalian species. Approximately 50% of pregnant women have an abnormally… (More)

This study assessed biotin nutritional status longitudinally during pregnancy as judged by urinary excretion of biotin and biotin… (More)

To assess the utility of various indicators of biotin status, marginal biotin deficiency was induced experimentally in normal… (More)

Using serum biotin concentration as the indicator, a previous study reported biotin deficiency resulting from long-term… (More)

This study describes a standardized method for recording single-pulse 1H-nuclear magnetic resonance (1H-NMR) spectra from… (More)

Two brothers, aged 7 and 5 years, who excreted large amounts of the leucine metabolites 3-methylglutaconic acid, 3-methylglutaric… (More)