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Biomedical subjects

O A Mamer

Publications and source records attributed to O A Mamer.

72 records · Page 4Linked to original sources

Profiles in altered metabolism. II.--Accumulation of homogentisic acid in serum and urine following acetylsalicylic acid ingestion.

Homogentisic acid elevations have been found in urine and serum of patients on long term heavy salicylate therapy and those acutely intoxicated with acetylsalicylic acid. Loading of two normal volunteers with single oral acetylsalicylate doses produced transient elevations in urinary homogentisic acid excretion. These findings suggest that heavy salicylate use results in the partial inhibition of homogentisic acid oxidase in vivo.

Aspirin↗

Demonstration of a new mammalian isoleucine catabolic pathway yielding an Rseries of metabolites.

1. Normal human urine contains small amounts (less than 4 mg/g of creatinine) of 2-ethylhydracrylic acid, formed, we believe, by a previously undisclosed endogenous catabolic pathway for the oxidation of a newly described series of R metabolites of isoleucine. 2. Urinary excretion of 2-ethylhydracrylic acid is variably increased in defects of isoleucine oxidation at distal steps in the catabolic pathway (3-oxoacyl-CoA thiolase deficiency and methylmalonyl-CoA mutase deficiency) and is diminished when proximal steps of the oxidative pathway are blocked as in branched-chain oxo acid decarboxylase deficiency ('maple-syrup-urine' disease). 3. Precursors of R-pathway metabolites [R(-)-2-methylbutyrate and 2-ethylacrylate ] lead to increased 2-ethylhydracrylate excretion in the mammal(rat, rabbit and dog); the corresponding S metabolites [S(+)-2-methylbutyric acid and tiglic acid ], when given in equimolar amounts, have little effect on its excretion, suggesting that little or no interconversion between S and R metabolites occurs in vivo. 4. Studies with 2H-labelled precursors indicate that conversion of R 2-methylbutyrate into 2-ethylhydracrylic acid occurs by a direct pathway (apparently via 2-ethylacrylic acid). 5. The further oxidation of 2-ethylhydracrylic acid to ethylmalonic acid was demonstrated, and may be analogous to S-metabolite oxidation via methyl malonate. 6. Valine metabolites do not interact with the R=isoleucine pathway under the conditions of these experiments in vivo.

Acrylates↗

Measurement of prostaglandin F2alpha levels in human cerebrospinal fluid in normal and pathological conditions.

Prostaglandin F2alpha concentrations were measured in human cerebrospinal fluid by the gas-chromatography-mass spectrometric technique using 2-H4-PGF2 alpha as internal standard and carrier. Normal levels of 71.6 plus or minus 34.7 pg/ml were found. Considerable increases in PGF2 alpha concentrations were found in patients with epilepsy, meningtitis or following cerebrovascular accidents or neurosurgical removals of brain tissue. The results agree in general with recent measurements using radioimmunoassay.

Central Nervous System Diseases↗

The syndrome of systemic carnitine deficiency. Clinical, morphologic, biochemical, and pathophysiologic features.

An 11-year old boy had had recurrent episodes of hepatic and cerebral dysfunction and underdeveloped musculature. Overt weakness developed at age 10. Lipid excess, especially in type I fibers, was found in muscle. Hypertrophied smooth endoplasmic reticulum and excessive microbodies were present in liver. Marked carnitine deficiency was shown in skeletal muscle, plasma, and liver. Ketogenesis was impaired on a high fat diet, but omega oxidation of fatty acids was enhanced. There was excessive glucose uptake and essentially no oxidation of labeled long-chain fatty acids by perfused forearm muscles in vivo. Oral replacement therapy restored plasma carnitine levels to normal, but not liver or muscle carnitine levels, and was accompanied by clinical improvement.

Adenosine Triphosphatases↗

A "new" disorder of isoleucine catabolism.

A disorder of isoleucine catabolism was found in a child with intermittent metabolic acidosis. The block occurs at the stage of propionate synthesis, and two intermediates--alpha-methylacetoacetate and alpha-methyl-beta-hydroxybutyrate--accumulate. The condition is apparently inherited.

Acetoacetates↗

Chemical and epidemiological aspects of modified butter oil fractions.

Butter lipids are an important traditional source of dietary energy intake in the form of fat. Butter lost a sizable portion of its market share due to controversies associated with its cholesterol content and high percentage of long-chain saturated fatty acids. Accordingly, the use of vegetable oils and their chemically manipulated counterparts such as those produced by partial hydrogenation or interestrification increased proportionally. However, beginning in 1940, researchers developed several procedures such as temperature-controlled crystallization, refractionation of crystallized butter oil solids, and supercritical carbon dioxide extraction to improve the acceptance of butter oil. Others proposed preparation of synthetic substitutes such as sucrose polyesters to reduce intestinal absorption of fatty acids, thus reducing caloric intake with concomitant reduction in serum cholesterol. The present review provides a summary of the efforts of several attempts to improve the acceptability of butter together with the anticipated epidemiological consequences of long-term consumption of altered butter oil to mammalian health.

Butter↗