Subject(s)
Creatine Kinase/blood , Reagent Kits, Diagnostic , Evaluation Studies as Topic , Humans , IsoenzymesABSTRACT
A procedure for estimating urine volumes from urine weights is presented. Regression analysis was used to assess correlation between weight-volume errors. The urine weight was divided by 1.016, the mean urine specific gravity in our patient population. It was found that the gravimetric method gave results lower than the volume method by one percent or less. We conclude that this difference is acceptable for clinical purposes. The gravimetric procedure has the advantages of rapidity, cost-effectiveness, and safety.
Subject(s)
Urine , Humans , Specimen Handling/methodsABSTRACT
The deficiency of oleic acid as one of the fatty acids in glucocerebrosides that accumulate (31--77 mg/g dry weight) in the spleen in patients with Gaucher's disease was confirmed in 9 cases. In an effort to account for the 10-fold difference between the oleoyl glycocerebroside content of glucocerebrosides in spleen from controls and patients with Gaucher's disease, we compared the ability of extracts of spleen and fibroblasts from individuals with various forms of Gaucher's disease and controls to hydrolyze [14C]stearoyl and [3H]oleoyl glucocerebroside. The residual glucosylceramidase activity in patients with Gaucher's disease hydrolyzes the glucose moiety of oleoyl glucocerebroside at approximately the same rate as that of stearoyl glucocerebroside. Similarly, the more active glucosylceramidase of control tissue acts upon both oleoyl and stearoyl glucocerebrosides with equal efficiency. These observations indicate that a mutation affecting the substrate specificity of glucosylceramidase cannot account for the lack of oleic acid-containing glucocerebrosides in patients with Gaucher's disease. Thus, the hypothesis that the difference in fatty acid composition found in glucocerebroside is obtained as a result of a mutation affecting the specificity of the residual glucosylceramidase must be rejected.
Subject(s)
Cerebrosides/metabolism , Gaucher Disease/metabolism , Glucosylceramides/metabolism , Adult , Fibroblasts/metabolism , Glucosylceramidase/metabolism , Humans , Hydrolysis , Infant , Oleic Acids/analysis , Spleen/enzymology , Spleen/metabolism , Stearic Acids/analysis , Substrate SpecificityABSTRACT
HeLa cells exposed to trace amounts of pentadecan-2-one showed changes in metabolism of 1(-14)C-palmitate. These changes consisted of an increased incorporation of radioactivity into the triglycerides and free fatty acids and a decreased 14C incorporation into the ether moiety of alk-1-enyl acyl phosphoglycerides. Chemical analysis of the several lipid fractions showed a threefold increase in triglyceride content but no change in the amount of alk-1-enyl acyl or diacyl phosphoglycerides in the treated cells. Pentadecan-2-one added to the culture medium apparently gains entrance to the cell since both pentadecan-2-one and pentadecan-2-ol were detected in the ketone-treated cells and their culture medium.
Subject(s)
Ketones/pharmacology , Lipid Metabolism , Fatty Acids, Nonesterified/metabolism , Fatty Alcohols/metabolism , HeLa Cells/metabolism , Palmitic Acids/metabolism , Phospholipids/metabolismABSTRACT
A disaccharide was isolated from rat mammary tissue and determined to be 6-O-beta-galactopyranosyl myo-inositol (6-beta-galactinol) on the basis of combined gas-liquid chromatography-mass spectrometry of the permethylated and peracetylated derivatives as well as previously reported chemical and enzymatic evidence. 6-beta-Galactinol, also found in rat milk, increased during lactation, and on the 18th day represented approximately 17% of the total non-lipid neutral myo-inositol. The sugar was absent in all other rat tissues examined, suggesting a unique association with the process of lactation. This novel galactoside of one human subject on the basis of paper and gas-liquid chromatography.
