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Thesis (Ph. D.)--University of Wisconsin--Madison, 1965. / Vita. Typescript. eContent provider-neutral record in process. Description based on print version record. Includes bibliographical references.

xvi, 227 leaves : ill. ; 26 cm. / Title page, contents and abstract only. The complete thesis in print form is available from the University Library. / Thesis (Ph.D.)--University of Adelaide, Dept. of Plant Physiology, 1971

Proefschrift--Utrecht. / Title also in English. Summary in English. Vita. Includes bibliographical references.

Proefschrift--Utrecht. / Title also in English. Summary in English. Vita. Includes bibliographical references.

Methylmalonic aciduria results from defects in the enzyme methylmalonyl-CoA mutase and from defects in the synthesis of the enzyme's cofactor adenosylcobalamin. Two patients who excrete methylmalonic acid have been shown to have a homozygous nonsense mutation in the methylmalonyl-CoA epimerase gene (MCEE). To further understand the causes of methylmalonic acid excretion, the MCEE gene was sequenced in 229 patients who excreted methylmalonic acid for which no cause was known. Mutations were detected in five patients. Fusion of fibroblast lines from two patients with a homozygous nonsense mutation in MCEE did not result in correction of [14C]propionate incorporation toward control values while the defect in these fibroblasts was complemented by mut, cblA, and cblB fibroblasts. Transfection with wild-type MCEE cDNA resulted in correction of the biochemical phenotype in cells from both patients. These experiments support the hypothesis that a defective epimerase enzyme can be a cause of elevated methylmalonic acid excretion.

Leukemia is the most common cancer of children, moreover it is also not uncommon of elderly patients. Research has focused on the development of specific antileukemic drugs in recent years. Abnormalities in tumor cell metabolism that can be targeted during treatment appear to be the key. Natural 1,4-naphthoquinones, including quambalarin B produced as a secondary metabolite by the basidiomycetes of Quambalaria cyanescens, are known for their therapeutic effects. Not surprisingly, Quambalarine B has also been shown to inhibit cell proliferation in some leukemic cell lines and subsequently caused cell death. In the present thesis, I tried to observe changes in amino acid metabolism by monitoring amino acid levels in the intracellular and extracellular environment of leukemic cells after treatment with Quambalarine B using amino acid analysis with fluorescence detection. The observation was performed in Jurkat, Ramos and THP-1 cell lines, each of these lines represents another type of leukemic disease. [IN CZECH] Key words Amino acid analysis, amino acid metabolism, Quambalarine B, leukemia

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