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Biomedical subjects

G A Mitchell

Publications and source records attributed to G A Mitchell.

At least 91 records · Page 5Linked to original sources
PubMed1986

Clinical heterogeneity in cobalamin C variant of combined homocystinuria and methylmalonic aciduria.

We describe two patients with methylmalonic aciduria and homocystinuria (Cbl C). The disorder was not diagnosed in patient 1 until 4 1/2 years of age; he had a history of fatigue, anorexia, delirium, and spasticity. Moderate megaloblastic bone marrow changes were observed, and there was hyperreflexia of the lower limbs. His condition improved clinically with hydroxycobalamin therapy. Patient 2 was hospitalized at 6 weeks of age because of lethargy and poor feeding. She was found to have macrocytosis. Despite an initial good clinical response to hydroxycobalamin, she developed a striking pigmentary retinopathy. Methylmalonic aciduria persisted in both patients, and homocystinuria persisted in patient 1 despite therapy. The diagnosis of Cbl C disease has been confirmed in both patients by biochemical studies of cultured fibroblasts, including complementation studies. The differences in age of onset and clinical findings together with the similar biochemical findings in these two patients demonstrate the heterogeneity of phenotypic expression in patients with apparently identical abnormalities of vitamin B12 metabolism.

Amino Acid Metabolism, Inborn Errors↗
PubMed1985

Novel rhodamine tripeptide substrate for manual and automated colorimetric prothrombin time test.

A chromogenic prothrombin time test is described using the tripeptide substrate, (Sar-Pro-Arg)2-Rhodamine 110. The method is both precise and sensitive to the factors of the extrinsic pathway. The accuracy of the method was demonstrated by comparison of patient sample values to standard clotting test results. The manual substrate test was adapted to two different automated chemistry analyzers.

Autoanalysis↗
PubMed1983

Sensitivity of two Chinese hamster cell lines to SCE induction by a variety of chemical mutagens.

SCE induction in Chinese hamster Don (lung) cells was compared with that in CHO (ovary) cells exposed under identical conditions to 14 known mutagens. Test protocols used for comparison were selected following a study of Don and CHO cell responses to aflatoxin B1 and benzo[a]pyrene. In the absence of added metabolizing enzymes 9-aminoacridine, 4-nitroquinoline 1-oxide, N-methyl-N-nitrosourea, dimethylcarbamoyl chloride, beta-propiolactone, daunomycin, aflatoxin B1 and 2-aminoanthracene were directly active in both cell lines; every substance positive in CHO cells was also positive in Don cells. However, the latter detected cyclophosphamide, hydrazine sulphate, benz[c]acridine, 3-methylcholanthrene and benzo[a]pyrene without addition of S9. CHO cells did not respond equivalently to these mutagens, either in the presence or absence of S9. Other differences between the cell lines depended on chemical exposure time, S9 pre-incubation or co-incubation conditions. For example, the ability of CHO cells to detect SCEs due to 2-aminoanthracene was acutely dependent on exposure time. In addition, Don cells exhibited lower background SCE values which were less variable than those of CHO cells under the same culture conditions. Although incapable of detecting 4-dimethylaminoazobenzene (butter yellow) and not as sensitive to cyclophosphamide as certain cell lines of liver origin, the pseudodiploid Don cell line possesses other desirable characteristics required for in vitro SCE assays, particularly with regard to intrinsic metabolic activation of polycyclic aromatic hydrocarbons and related substances.

Aflatoxin B1↗
PubMed1982

Sister chromatid exchange induction in two cell lines.

Sister chromatid exchange (SCE) induction by the procarcinogen benzo (a)-pyrene (BP) was studied using two established cell lines. Chinese hamster ovary (CHO) and Chinese hamster lung (Don) cells were exposed to varying concentrations of BP in the presence and absence of a rat liver metabolizing system. Results showed a significant difference in the abilities of the two cell lines to induce SCE in the absence of liver homogenate. These experiments demonstrate that Don cells are capable of generating active metabolites from biologically inactive procarcinogens presumably due to an inherent metabolizing system which is deficient or absent in CHO cells.

Animals↗
PubMed1982

Gas-liquid chromatographic determination of adipate content of acetylated di-starch adipate.

A gas-liquid chromatographic method is described for rapid, quantitative determination of adipate content of acetylated di-starch adipate. The adipate group is very labile and, under mild alkaline conditions at ambient temperature, is easily hydrolyzed from the starch. Free adipic acid is formed by acidification of the solution with HCl, and then extracted with ethyl acetate. Ethyl acetate is removed under vacuum distillation, and a silyl derivative of the adipic acid is formed. Glutaric acid internal standard is introduced into the original starch sample before hydrolysis. An aliquot of the silylated solution is injected into a gas chromatograph fitted with a column having silicone oil as the active phase. A flame ionization detector is also incorporated. Results correlate well the amount of adipylating reagent used. No adipic acid is detectable when a hydrolyzed, extracted sample of acetylated di-starch adipate is subjected to a second extraction. Recovery levels of adipic acid, from starches fortified with 100-500 ppm, are in the range of 97-102.5%.

Adipates↗
PubMed1976

Decreased radioassay values for folate after serum extraction when pteroylglutamic acid standards are used.

Radoiassay values for folate in protein-denautured (extracted) sera are compared with values obtained by individual serum blank correction. Lower folate values are reported for sera extracted at pH 1.3, 7.6, 10.5, and 12.4 when pterolyglutamic acid (PGA) calibration standards are used. The results are unchanged when ascorbate added to the sera is increased from 1 to 20 g/liter and when [3H]PGA is substituted for 125I-labeled PGA. However, a higher mean value for extracted serum is reported when N-5-methyltetrahydrofolic acid calibration standards are substituted for PGA. Because both patients' samples and assay calibrators were subjected to the same extraction methods, our results suggest that serum folate behaves differently from PGA during the extractions.

Folic Acid↗