Profiling of human body fluids in healthy and diseased states using gas chromatography and mass spectrometry, with special reference to organic acids
Journal of Chromatography B Biomedical Sciences and ApplicationsPublished 1 September 1977
Egil Jellum
Citations210
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TL;DR
Stable isotopes and gas chromatography--mass spectrometry are suitable for quantitative analysis of many compounds in the body fluids, and well suited for investigation of metabolic pathways.
Abstract
This review summarizes recent advances in the application of gas chromatography and mass spectrometry to the study of human diseases. Emphasis is placed upon the organic acid profiles of the various body fluids. Methods for sample work-up prior to separation and mass spectrometric analysis are reviewed, and artifacts and pitfalls are discussed. Organic acid profiles, obtained with packed or capillary columns attached to mass spectrometers with or without computer systems, have led to the discovery of new normal metabolites, new metabolic disorders, and to new knowledge about a number of other diseases. Stable isotopes and gas chromatography--mass spectrometry are suitable for quantitative analysis of many compounds in the body fluids, and well suited for investigation of metabolic pathways.
Keywords
ChemistryBiochemistry, Genetics and Molecular Biology
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Journal of Chromatography B Biomedical Sciences and ApplicationsInvestigation of the metabolic pattern in maple syrup urine disease by means of glass capillary gas chromatography and mass spectrometry
49 Citations1977Cornelis Jakobs, E. Solem +3 more
The results clearly demonstrate that new information on the metabolic situation in well known disorders may be obtained by exploiting the high resolving power of capillary columns.
Scandinavian Journal of Clinical and Laboratory InvestigationClinical Conditions Associated with Urinary Excretion of 2-Hydroxybutyric Acid
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It is suggested that the large amounts of glycine found in various body fluids are secondary to a hitherto undescribed enzymatic defect in the degradation of D-glyceric acid.
Clinica Chimica ActaD-Glyceric acidemia in a patient with chronic metabolic acedosis
47 Citations1976S.K. Wadman, M. Durán +9 more
The glyceric acid was found to have the D-configuration, as analyzed by capillary gas chromatography of its di-O-acetyl-l-menthyl ester, which may result from a defect in serine metabolism.
Clinical ChemistryIon chromatography: a new technique for clinical chemistry.
47 Citations1976Carolyn E. Anderson
Ion chromatography is shown to be a practical analytical tool, particularly for ions having no chromophores, such as sodium, ammonium, potassium, magensium, calcium, chloride, nitrite, phosphate, and sulfate.
Clinica Chimica ActaQuantitative studies on the urinary excretion of unconjugated aromatic acids in phenylketonuria
46 Citations1974R. A. Chalmers, R. W. E. Watts
The study of a wide range of biochemical parameters may improve the ability to differentiate the atypical forms of phenylketonuria.
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46 Citations1974Gerald Lancaster, Orval Mamer +1 more
Thiamine-responsive MSUD is characterized by greater accumulation of α-keto-B-methyl-valerate relative to the two other branched-chain keto acids when compared with keto acid ratios in classical MSUD, and thiamine preferentially stimulates the oxidation of isoleucine and its keto Acid in cultured skin fibroblasts.
Clinical ChemistryUrinary organic acids in man. III. Quantitative ranges and patterns of excretion in a normal population.
46 Citations1976R. A. Chalmers, M. J. R. Healy +3 more
The quantitative valuse observed enable the relative significance of different urinary anionic metabolites to be assessed, including the recently identified aldonic and deoxyaldonic acids.
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46 Citations1963Charles W. Gehrke, Donald F. Goerlitz
Clinica Chimica Acta3-Hydroxy-3-methylglutaric aciduria, combined with 3-methylglutaconic aciduria
45 Citations1976S.J. Wysocki, Steven Wilkinson +3 more
The profile of acids in urine indicates a blockage in the sixth step of leucine catabolism, the cleavage of 3-hydroxy-3-methylglutaryl- CoA to acetoacetic acid and acetyl-CoA.
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44 Citations1976Kym F. Faull, G M Schier +2 more
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BMJSpecific Immune Response in Human Skin Carcinoma
44 Citations1971R. C. Nairn, A. P. P. Nind +4 more
Eight out of nine patients with squamous cell carcinoma of skin have shown immunological reactivity against their own tumour cells by one or more tests with their sera or peripheral blood lymphocytes, and, with cultured tumour, complement-dependent serum cytotoxicity and lymphocyte attack.
Journal of Chromatography AProfiles of volatile metabolites in body fluids
43 Citations1976M. Stafford, M. G. Horning +1 more
A method for the analysis of volatile metabolites present in plasma, urine, breast milk and amniotic fluid collected from mother-infant pairs has been developed which requires only 100 mul of plasma, 3 ml of urine, 20 mul of breast Milk and 500 mul of amniotics fluid.
Journal of Chromatography B Biomedical Sciences and ApplicationsRoutine chromatography of simple lipids and their constituents
43 Citations1977A. Kuksis
This review summarizes the basic chromatographic routines commonly employed in lipid research laboratories in the analysis of the lipid mixtures normally isolated from natural sources to show that for many purposes adequate analyses of known lipids can be obtained with conventional equipment of thin-layer and gas chromatography.
Clinical ChemistryProtein-bound carbohydrates in breast cancer. Liquid-chromatographic analysis for mannose, galactose, fucose, and sialic acid in serum.
43 Citations1976J.E. Mrochek, Stanley R Dinsmore +2 more
Sera from breast-cancer patients whose malignancy had been categorized as either stable, responsive, or progressive (based on clinical observations spaced from two to five months apart) were assessed, with use of a cerate oxidimetric detector.
Clinical ChemistryStructure of some aliphatic dicarboxylic acids found in the urine of an infant with congenital lactic acidosis.
43 Citations1976Sandra Lindstedt, K. Norberg +2 more
It is proposed that impaired beta-oxidation, probably at the acyl-CoA-dehydrogenase step, resulted in the formation of the observed acids, and suggests a genetic defect.
Pediatric ResearchMetabolic Studies of a Family with Massive Formiminoglutamic Aciduria
42 Citations1975Thomas L. Perry, Derek A. Applegarth +3 more
Two siblings who excrete massive amounts of N-formiminoglutamic acid (FIGLU) in their urine presumably have a deficiency in activity of the hepatic enzyme, glutamate formiminotransferase, which carries out the fourth sequential step in the main pathway of histidine degradation.
Clinical ChemistryProfiles of ultraviolet-absorbing components of urine from autistic children, as obtained by high-resolution ion-exchange chromatography.
42 Citations1976Adam W. Lis, Iris G. McLaughlin +3 more
Urines from 19 autistic children were analyzed by high-resolution, high-pressure anion-exchange chromatography and showed abnormalities in the excretion of hippuric acid, 4-hydroxyhippuric Acid, and N1-methyl-2-pyridone-5-carboxamide, the end product of the nicotinic acid pathway.
Clinica Chimica ActaThe occurrence of 2-hydroxybutyric acid in urine from patients with lactic acidosis
42 Citations1973Jon Elling Pettersen, Sverre Landaas +1 more
It is concluded that this metabolite is probably formed by lactate dehydrogenase from 2-oxobutyrate, a normal intermediate in the metabolism of some amino acids.
Clinica Chimica ActaGas chromatographic determination of urinary vanilglycolic acid, vanilglycol, vanilacetic acid and vanillactic acid — Chemical parameters for the diagnosis of neurogenic tumours and the evaluation of their treatment
41 Citations1976S.K. Wadman, D. Ketting +1 more
A gas chromatographic method is described for the determination of urinary vanilglycolic acid,Vanilgly col, vanilacetic acid and vanillactic acid as their trimethylsilyl derivatives, which are chemical parameters for the dignosis of neurogenic tumours.
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