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At least 991 records · Page 55Linked to original sources
PubMed1996

Distinction of dicarboxylic aciduria due to medium-chain triglyceride feeding from that due to abnormal fatty acid oxidation and fasting in children.

Increased amounts of dicarboxylic acids are excreted in human urine under conditions of medium-chain triglyceride (MCT) feeding, abnormal fatty acid oxidation (FAO) and fasting. Criteria to distinguish dicarboxylic aciduria originating from MCT feeding and other conditions are needed in urinary organic acid profiling for detecting inborn errors of metabolism. Patterns of dicarboxylic aciduria in children under various conditions were compared. The relative amounts of medium-chain saturated dicarboxylic acids in urine are not reliable for identifying MCT-induced dicarboxylic aciduria. On the other hand, low ratios of unsaturated to saturated dicarboxylic acids (<0.1) and 3- hydroxydecenedioic to 3-hydroxydecanedioic acids were found to be useful in identifying dicarboxylic aciduria due to MCT ingestion. Additional unique features of dicarboxylic aciduria from MCT are low ratios of 3-hydroxydodecanedioic to 3-hydroxydecanedioic acid (<0.14) and 3-hydroxyadipic to adipic acid (<0.02).

Caproates↗
PubMed1997

Splanchnic versus whole-body production of alpha-ketoisocaproate from leucine in the fed state.

The extent to which dietary branched-chain amino acids are deaminated by the splanchnic tissues (ie, the liver and gut) in the fed state and released as ketoacids into the systemic circulation is not known. To determine this, we combined the oral (L-[1-13C]-leucine, [13C]-Leu) and intravenous (L-[5,5,5-2H3]leucine, [2H3]-Leu) leucine tracer infusion with the intravenous administration of an independent isotope of the leucine ketoanalog alpha-ketoisocaproic acid (KIC) ([4,5-3H]KIC). The study was conducted during constant administration of a complete mixed meal. We found that 26% +/- 5% of the orally administered leucine was taken up by the splanchnic organs at first pass, whereas 74% +/- 5% appeared in the systemic circulation. The rate of splanchnic KIC release from deamination of dietary leucine accounted for 3% +/- 0.2% of the oral leucine administration rate and 13% +/- 2% of leucine splanchnic uptake (fractional splanchnic deamination). The fraction of whole-body total leucine uptake that was deaminated to KIC was 41% +/- 5% (P < .05 v fractional splanchnic deamination of dietary leucine uptake). We conclude that (1) the release of KIC from leucine deamination within splanchnic tissues constitutes a minimal fraction of first-pass dietary leucine uptake, and (2) splanchnic tissues are relatively less efficient than the whole body in KIC production from leucine deamination.

Adult↗
PubMed1981

New assays for the enzymatic conversion of cholesterol to pregnenolone.

Cholesterol side chain cleavage is determined by means of separation of (26-C14)-cholesterol and its radioactively labeled side chain (1-C14)k-isocaproic acid. Alumina minicolumn assay (AMCA): adsorption of cholesterol from an aqueous phase by aluminium oxide, while isocaproic acid can percolate through the column. In modification of a previously described technique (1), cholesterol is quantitatively eluted by ethanol. Filter assay (FA): retention of cholesterol by a membrane filter (pore size less than or equal to 0.1 um) while isocaproic acid can pass the filter. Two-phase scintillation assay (TPSA): pH-dependent partition of isocaproic acid between an organic scintillation mixture and an aqueous phase. The TPSA can be applied for all enzymatic reaction in which the polarity of the radioactive residue which is split off depends on pH values or when the total charge of a polar molecule is changed to an apolar state by cleaving one non-radioactive group (e.g. steroid sulfates) and vice versa. The criteria or reliability of the test systems are described. Bovine adrenal mitochondria were incubated and the side chain cleavage of (26-C14)-cholesterol was studied by the new tests systems and compared to the conversion rates of (4-C14)-cholesterol to its metabolites are determined by thin layer chromatography. A good agreement of all tests was found.

Aldehydes↗
PubMed2002

Interactive effects of the electron acceptor sulphate and o-cresol on the methanogenic degradation of hexanoate.

A three-stage continuous culture system was used to segregate the component microbial groups of a methanogenic hexanoate-degrading association enriched from anaerobic refuse. The inhibitory effects of o-cresol concentrations (2-20 mM) on the fermentative, acetogenic, sulphate-reducing and methanogenic bacteria were then assessed in the presence of either 1.4 or 3.5 mM sulphate in the influent medium. The sulphate-reducing bacteria (SRB) in the 1.4 mM sulphate-supplemented systems were the most sensitive to o-cresol, with 29.3 and 56.6% inhibition on supplementation with 4 and 6 mM o-cresol, respectively. With 3.5mM supplementation, inhibition was 4.5 and 19.4%, respectively. Methanogenesis was not inhibited by concentrations < 10 mM o-cresol, and complete inhibition was recorded only at concentrations > or = 10 mM. Both fermentation and acetogenesis were affected by inhibition of the electron sinks. The increase in influent sulphate concentration promoted electron flow to sulphidogenesis, as predicted on thermodynamic criteria, but did not affect the relative sensitivity of the different physiological groups.

Bacteria, Anaerobic↗
PubMed2001

A review of the environmental fate and aquatic effects of a series of C4 and C8 oxo-process chemicals.

Environmental fate and aquatic effects data were examined for a series of C4 (butyl acetate, 1-butanol, isobutyl alcohol) and C8 (2-ethylhexanol and 2-ethylhexanoic acid) oxo-process chemicals. Manufacturing of these chemicals requires enclosed equipment, so environmental releases are generally limited to volatilization during their use, handling or transport. C4 compounds are more soluble and volatile, and would bind to soil and sediment to a lesser extent than C8 compounds. All five compounds were readily biodegradable based on OECD and APHA tests conducted up to 28 days. Atmospheric photo-oxidation half-lives range from 0.43 to 3.8 days. Toxicity data show that all five compounds pose generally low concern to fish, invertebrates, algae, and microorganisms. Overall, the data show that inadvertent releases of these compounds into the environment would be rapidly biodegraded in soil and water, volatilize to the atmosphere subject to photo-oxidation, while any residues remaining in water would pose a negligible threat to aquatic life.

1-Butanol↗
PubMed1977

Renin.

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Animals↗