Chiral compounds as indicators of inherited metabolic disease. Simultaneous stereodifferentiation of lactic-, 2-hydroxyglutaric- and glyceric acid by enantioselective cGC.
Enantiomer
; 1(3): 177-82, 1996.
Article
en En
| MEDLINE
| ID: mdl-9676282
Urinary organic acid analysis is a vital diagnostic tool in the investigation of patients with suspected inborn errors of metabolism. Recent descriptions of diseases producing abnormal chiral metabolites have provided the need for individual enantiomer analysis. In this paper the isolation and simultaneous stereodifferentiation of lactic, glyceric and 2-hydroxyglutaric acids using capillary gas chromatography on heptakis (2,3-di-O-methyl-6-O-tert.butyldimethylsilyl)-beta-cyclodextrin as the chiral stationary phase is described. The diagnostic potential of this method is demonstrated by the analysis of urine samples from two patients with D-2-hydroxyglutaric aciduria and a dog with D-glyceric aciduria. Due to the lack of specific clinical symptoms in these diseases, enantiomeric analysis is vital for diagnosis enabling prompt therapeutic intervention.
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Colección:
01-internacional
Base de datos:
MEDLINE
Asunto principal:
Ácido Láctico
/
Enfermedades de los Perros
/
Glutaratos
/
Ácidos Glicéricos
/
Errores Innatos del Metabolismo
Tipo de estudio:
Diagnostic_studies
Límite:
Animals
/
Child, preschool
/
Female
/
Humans
/
Infant
/
Male
Idioma:
En
Revista:
Enantiomer
Asunto de la revista:
BIOQUIMICA
Año:
1996
Tipo del documento:
Article
País de afiliación:
Alemania
Pais de publicación:
Estados Unidos