N-carbamylglutamate augments ureagenesis and reduces ammonia and glutamine in propionic acidemia.

ABSTRACT:

Objectives

The objective of this study was to determine whether N-carbamylglutamate (NCG) reduces plasma levels of ammonia and glutamine and increases the rate of ureagenesis in patients with propionic acidemia (PA).

Methods

Identical 4-hour studies were performed before and immediately after a 3-day trial of oral NCG in 7 patients with PA. An oral bolus of [(13)C]sodium acetate was administered at the start of each study, and sequential blood samples were obtained to measure [(13)C]urea, ammonia, urea, and amino acids.

Results

With longitudinal mixed-effects linear regression, peak [(13)C]urea increased after treatment with NCG (from 2.2 to 3.8 microM; P < .0005). There were concomitant decreases in mean plasma ammonia (59-43 microM; P < .018) and glutamine (552-331

SUBMITTER: Ah Mew N 

PROVIDER: S-EPMC3297024 | biostudies-literature | 2010 Jul

REPOSITORIES: biostudies-literature