Microbiological test for leucine, valine, and isoleucine using urine sample dried on filter paper.
نویسندگان
چکیده
Urine specimens dried on filter paper squares were obtained from 700 mentally retarded children. A microbiological assay was used to screen the specimens for the presence of valine, leucine, and isoleucine. Three specimens gave positive tests for all three amino acids. A generalized aminoaciduria was observed in two specimens, and in the third, elevation of valine and leucine/isoleucine was confirmed by paper chromatography. The use of these screening procedures for early detection of children with maple sugar urine disease is suggested.
منابع مشابه
Diagnosis of maple syrup urine disease by determination of L-valine, L-isoleucine, L-leucine and L-phenylalanine in neonatal blood spots by gas chromatography-mass spectrometry.
A novel method was developed for the diagnosis of maple syrup urine disease (MSUD) by the determination of L-valine, L-leucine, L-isoleucine and L-phenylalanine in dried blood spots of newborns by gas chromatography-mass spectrometry (GC-MS). The four amino acids were extracted from blood samples by methanol and derivatized by n-butanol and trifluroacetic anhydride under optimum reaction condit...
متن کاملStudies on amino acid excretion in man; amino acids in urine.
Recent interest in the r81e of proteins in gastrointestinal disease has prompted an investigation of certain aspects of this problem. Studies by Cannon and coworkers suggested to us that measurements of the intake and excretion of individual amino acids might supply more complete and significant data than the conventional nitrogen balances alone (1). Hence, in addition to the nitrogen balance, ...
متن کاملSecond-tier test for quantification of alloisoleucine and branched-chain amino acids in dried blood spots to improve newborn screening for maple syrup urine disease (MSUD).
BACKGROUND Newborn screening for maple syrup urine disease (MSUD) relies on finding increased concentrations of the branched-chain amino acids (BCAAs) leucine, isoleucine, and valine by tandem mass spectrometry (MS/MS). d-Alloisoleucine (allo-Ile) is the only pathognomonic marker of MSUD, but it cannot be identified by existing screening methods because it is not differentiated from isobaric am...
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Newborn screening for MSUD is a special challenge since patients with MSUD can metabolically decompensate rapidly without adequate treatment within the first two weeks of life. However, the screening method does not detect the actual marker metabolite (alloisoleucine) specifically, but only as part of the group of the other isobaric amino acids leucine, isoleucine and hydroxyproline. We describ...
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On the current study the effects of reducing branched-chain amino acids (BCAA) in diets of Sobaity sea bream in a constant level (40%) on growth and nutritional indices, pancreatic enzymes activity, whole body chemical proximate and amino acids, was assessed. This experiment was conducted in Marine Fish Research Station of Imam Khomeini harbor during July and August of 2014. For these purposes,...
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ورودعنوان ژورنال:
- Clinical chemistry
دوره 8 شماره
صفحات -
تاریخ انتشار 1962