Background: Maple syrup urine disease (MSUD) is an inherited branched-chain amino acid metabolic disorder caused by the deficiency in the branched-chain alpha-keto acid dehydrogenase (BCKD) complex. In MSUD, elevation of the branched-chain amino acids, such as alpha-keto acid and alpha-hydroxy acid, occurs due to the BCKDC gene deficiency, appearing in the blood, urine, and cerebrospinal fluid,...

OBJECTIVE To characterize the metabolic products of urine associated with preterm birth, thus providing clinical guidelines for intestinal and parenteral nutrition in preterm infants. METHODS Urine samples of 47 preterm infants and 45 full-term infants were collected and prepared for trimethylsilylation by treatment with urease. The levels of lysine, phenylalanine, histidine, ornithine, fumar...

ADDRESS: 219 Bryant Street, Buffalo 22, New York. V ARLETLES of syndromes have been described in which an abnormal amount of organic acid appears in the urine. In many of those syndromes which have been carefully studied, amino acids appear to constitute the major portion of organic acids. This probably is the result of the development of adequate methodology for the determination of amino acid...

Less information is available on the metabolism of organic arsenicals compared to inorganic arsenic in mammals. In the present study, we investigated tissue distribution, metabolism and excretion in rats of organoarsenicals, dimethylarsinic acid (DMAV), arsenobetaine (AB), arsenocholine (AC) and trimethylarsine oxide (TMAOV). Among these animals, arsenic concentrations in red blood cells (RBCs)...

We have previously identified proteins extracted from calcium oxalate (CaOx) and calcium phosphate (CaP) crystals generated experimentally in vitro in whole urine of healthy controls and stone formers. No significant differences were detected between protein components in matrices of crystals obtained from both groups. The aim of the present study was to estimate the amounts of six proteins ide...

low molecular weight organic acids are most predominant in apple juice among these components fumaric acid was not exceed more than 5 ppm. it was thought that the hplc is the best method of the determination of organic acids in the apple juice. in this study, chromatographic separation of organic acids of apple juice was obtained by preparing a sample and by applying them to acid phase for extr...

There is abundant evidence that glycosaminoglyeans are linked covalently to the protein in tissues (Mathews & Lozaityte, 1958; Muir, 1958). This fact suggested that isolation and characterization of glycosaminoglycan-peptides, particularly uronic acid-containing glycopeptides in normal urine, will assist in the understanding of the normal metabolism of glycosaminoglycans in tissues. The present...

Inorganic and organic mercury was measured in the red cells, plasma, and urine of five laboratory workers to examine the effect of D-penicillamine. The workers had had no particular exposure to mercurials. Before and during administration of D-penicillamine, inorganic mercury levels in plasma were significantly correlated with those in urine or 24-hour urinary outputs, but no significant correl...

BACKGROUND Stability of chemical speciation during sample handling and storage is a prerequisite to obtaining reliable results of trace element speciation analysis. There is no comprehensive information on the stability of common arsenic species, such as inorganic arsenite [As(III)], arsenate [As(V)], monomethylarsonic acid, dimethylarsinic acid, and arsenobetaine, in human urine. METHODS We ...