Urinary dicarboxylic acids in X-linked adrenoleukodystrophy.
The urinary excretion of dicarboxylic acids in X-linked adrenoleukodystrophy (X-ALD) was studied. The dicarboxylic acid profile in X-ALD did not show any specific pattern, unlike Zellweger syndrome and neonatal adrenoleukodystrophy. Medium-chain dicarboxylic acids with an even number of carbon atoms (adipic, suberic, sebacic) and an odd number of carbon atoms (pimelic, azelaic) were excreted within the normal ranges. Dicarboxylic acids with more than 10 carbon atoms were not found. These findings may be due to the normal beta-oxidation system of dicarboxylic acids in X-ALD.
Kodama
S
S
Department of Pediatrics, Himeji Red Cross Hospital, Hyogo, Japan.
Nishio
H
H
Nakajima
T
T
Nakamura
H
H
D000293:Adolescent; D000326:Adrenoleukodystrophy / Q000652:urine*; D002648:Child; D002675:Child, Preschool; D003998:Dicarboxylic Acids / Q000652:urine*; D008401:Gas Chromatography-Mass Spectrometry; D006801:Humans; D010084:Oxidation-Reduction
D003998:Dicarboxylic Acids