Level of translatable messenger RNA coding for argininosuccinate synthetase in the liver of the patients with quantitative-type citrullinemia |
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Authors: | Mariko Sase Keiko Kobayashi Yasushi Imamura Takeyori Saheki Kyoko Nakano Satoshi Miura Masataka Mori |
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Affiliation: | (1) Department of Biochemistry, School of Medicine, Kagoshima University, Usuki-cho 1208-1, 890 Kagoshima, Japan;(2) Department of Nutritional Biochemistry, Kagoshima Women's College, Murasakibaru 1-59-1, 890 Kagoshima, Japan;(3) Department of Bochemistry, School of Medicine, Chiba University, Inohana 1-8-1, 280 Chiba, Japan |
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Abstract: | Summary The translation activity of mRNA coding for argininosuccinate synthetase in total RNA extracted from the liver of three patients with quantitative-type citrullinemia was determined using a cell-free translation system. In two patients, the hepatic content of the enzyme was about 20% of the control value, whereas translatable mRNA level for the enzyme was similar to or slightly lower than those of control livers. In the third patient, the enzyme content was about 50% of the control value, and mRNA activity for the enzyme was low normal. These results indicate that at least in the first two patients, the decrease in the enzyme protein is due either to increased degradation of the enzyme or to decreased translation in the patient's liver. |
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