Argininosuccinate synthetase activity in cultured human lymphocytes
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چکیده
منابع مشابه
Argininosuccinate synthetase activity and citrulline metabolism in cells cultured from a citrullinemic subject.
Citrullinemia-is one of several human diseases caused by a defect in the KrebsHenseleit urea cycle. In this disorder a deficiency of liver argininosuccinate synthetase has been reported.' 1\Iorrow and his colleagues have recently described an infant with this condition2 and a fibroblast-like cell line has been derived from a skin biopsy of this child. In studying minimal media requirements for ...
متن کاملMolecular analysis of argininosuccinate synthetase deficiency in human fibroblasts.
We have analyzed cultured skin fibroblasts derived from patients with argininosuccinate synthetase deficiency for alterations in gene structure, mRNA content, and protein structure. Genomic DNA was digested with the endonucleases EcoRI or HindIII, and the fragments were analyzed by Southern blotting and hybridization with a cDNA probe for argininosuccinate synthetase. The blot pattern is comple...
متن کاملNitro analogs of substrates for argininosuccinate synthetase and argininosuccinate lyase.
The nitro analogs of aspartate and argininosuccinate were synthesized and tested as substrates and inhibitors of argininosuccinate synthetase and argininosuccinate lyase, respectively. The Vmax for 3-nitro-2-aminopropionic acid was found to be 60% of the maximal rate of aspartate utilization in the reaction catalyzed by argininosuccinate synthetase. Only the nitronate form of this substrate, in...
متن کاملExpression of the human argininosuccinate synthetase gene in hamster transferents.
The structural gene for human argininosuccinate synthetase [L-citrulline:L-aspartate ligase (AMP-forming), EC 6.3.4.5] was transferred to argininosuccinate synthetase-deficient Chinese hamster cells via metaphase chromosomes isolated from human lymphoblast line MGL8D1, a constitutive overproducer of argininosuccinate synthetase, and from its repressible parent, MGL8B2. Argininosuccinate synthet...
متن کاملRegulation of glucocorticoids of arginase and argininosuccinate synthetase in cultured rat hepatoma cells.
We have examined and characterized the regulation by glucocorticoids of the levels of arginase and argininosuccinate synthetase in two rat hepatoma cell lines (H4-II-E-C3 and MH1C1). Hydrocortisone elevates the activity of both enzymes in a time- and dose-dependent fashion. This effect was blunted markedly by small amounts of ethanol (0.1 to 0.9% [v/v]) and blocked substantially by a high molar...
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ژورنال
عنوان ژورنال: Biochemical Genetics
سال: 1977
ISSN: 0006-2928,1573-4927
DOI: 10.1007/bf00484469