A convenient enzymatic method for the determination of 4-methyl-2-oxopentanoate in plasma: comparison with high performance liquid chromatographic analysis.
A simple and rapid spectrophotometric method for the estimation of 4-methyl-2-oxopentanoate in plasma samples by use of NAD+-dependent D-2-hydroxyisocaproate dehydrogenase from Lactobacillus casei ssp. pseudoplantarum is described. It is based on the kinetic measurement of the decrease of NADH absorbance at 334 nm. Applicability is demonstrated by comparative measurement of 4-methyl-2-oxopentanoate content in plasma of patients with maple syrup urine disease by the enzymatic and a reversed phase high performance liquid chromatographic method.
Schadewaldt
P
P
Diabetes Forschungsinstitut, Universität Düsseldorf, FRG.
Hummel
W
W
Trautvetter
U
U
Wendel
U
U
D000429:Alcohol Oxidoreductases; D002851:Chromatography, High Pressure Liquid; D006801:Humans; D007651:Keto Acids / Q000097:blood*; D007780:Lacticaseibacillus casei / Q000201:enzymology; D008375:Maple Syrup Urine Disease / Q000097:blood; D008722:Methods; D009243:NAD; D013050:Spectrometry, Fluorescence
D007651:Keto Acids; D009243:NAD; C013082:alpha-ketoisocaproic acid; D000429:Alcohol Oxidoreductases; C047961:2-hydroxyisocaproate dehydrogenase