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New conjugated urinary metabolites in intermediate type maple syrup urine disease.

Clinica chimica acta; international journal of clinical chemistry (1987-10-30)
L Hagenfeldt, A S Naglo
PMID3677437
RESUMEN

N-Lactylvaline, N-lactylleucine, N-lactylisoleucine and the N-2-hydroxyisovaleryl conjugates of glycine, valine, leucine and isoleucine have been identified in urine from a patient with the intermediate type of maple syrup urine disease.

MATERIALES
Número de producto
Marca
Descripción del producto
219835
Sigma-Aldrich
2-Hydroxy-3-methylbutyric acid, 99%
En este momento no podemos mostrarle ni los precios ni la disponibilidad
379093
Sigma-Aldrich
(S)-(+)-2-Hydroxy-3-methylbutyric acid, 99%, optical purity ee: 99% (GLC)
En este momento no podemos mostrarle ni los precios ni la disponibilidad
55452
Sigma-Aldrich
D-α-Hydroxyisovaleric acid, ≥98.0% (T)
En este momento no podemos mostrarle ni los precios ni la disponibilidad