Abstract
Rat liver ornithine carbamoyltransferase appears to be located exclusively in the mitochondria; the activity that is found in the soluble fraction is indistinguishable from mitochondrial ornithine carbamoyltransferase by simple kinetic criteria, and seems to result from breakage of mitochondria during homogenization. Of several rat tissues studied, only the liver and the mucosa of small intestine contain significant amounts of ornithine carbamoyltransferase; the activity in intestinal mucosa is less than one thousandth of that in liver. Qualitatively, this distribution coincides with that of carbamoyl phosphate synthetase I and its cofactor, acetylglutamate. The rat liver contents of carbamoyl phosphate and ornithine were 0.1 and 0.15μmol/g wet wt. of tissue respectively. On the basis of these values, it is proposed that in vivo the ornithine carbamoyltransferase activity of liver may be much lower than its maximal activity in vitro might suggest.
Full text
PDFSelected References
These references are in PubMed. This may not be the complete list of references from this article.
- Adair L. B., Jones M. E. Purification and characteristics of aspartate transcarbamylase from Pseudomonas fluorescens. J Biol Chem. 1972 Apr 25;247(8):2308–2315. [PubMed] [Google Scholar]
- Arashima S., Matsuda I. Ornithine transcarbamylase, an isoelectric point (pI) isozyme in human liver and its deficiency. Biochem Biophys Res Commun. 1971 Oct 1;45(1):145–150. doi: 10.1016/0006-291x(71)90062-3. [DOI] [PubMed] [Google Scholar]
- Bourget P. A., Natale P. J., Tremblay G. C. Pyrimidine biosynthesis in rat liver: studies on the source of carbamoylphosphate. Biochem Biophys Res Commun. 1971 Nov;45(4):1109–1114. doi: 10.1016/0006-291x(71)90452-9. [DOI] [PubMed] [Google Scholar]
- CARAVACA J., GRISOLIA S. Synthesis of citrulline with animal and bacterial enzymes. J Biol Chem. 1960 Mar;235:684–693. [PubMed] [Google Scholar]
- Cathelineau L., Saudubray J. M., Polonovski C. Ornithine carbamyl transferase: the effects of pH on the kinetics of a mutant human enzyme. Clin Chim Acta. 1972 Oct;41:305–312. doi: 10.1016/0009-8981(72)90525-6. [DOI] [PubMed] [Google Scholar]
- Gamble J. G., Lehninger A. L. Transport of ornithine and citrulline across the mitochondrial membrane. J Biol Chem. 1973 Jan 25;248(2):610–618. [PubMed] [Google Scholar]
- Guthöhrlein G., Knappe J. Modified determination of citrulline. Anal Biochem. 1968 Oct 10;26(1):188–191. doi: 10.1016/0003-2697(68)90045-6. [DOI] [PubMed] [Google Scholar]
- Hager S. E., Jones M. E. Initial steps in pyrimidine synthesis in Ehrlich ascites carcinoma in vitro. II. The synthesis of carbamyl phosphate by a soluble, glutamine-dependent carbamyl phosphate synthetase. J Biol Chem. 1967 Dec 25;242(24):5667–5673. [PubMed] [Google Scholar]
- JONES M. E., ANDERSON A. D., ANDERSON C., HODES S. Citrulline synthesis in rat tissues. Arch Biochem Biophys. 1961 Dec;95:499–507. doi: 10.1016/0003-9861(61)90182-5. [DOI] [PubMed] [Google Scholar]
- Kemp J. W., Woodbury D. M. Synthesis of urea-cycle intermediates from citrulline in brain. Biochim Biophys Acta. 1965 Nov 15;111(1):23–31. doi: 10.1016/0304-4165(65)90469-1. [DOI] [PubMed] [Google Scholar]
- Levin B., Abraham J. M., Oberholzer V. G., Burgess E. A. Hyperammonaemia: a deficiency of liver ornithine transcarbamylase. Occurrence in mother and child. Arch Dis Child. 1969 Apr;44(234):152–161. doi: 10.1136/adc.44.234.152. [DOI] [PMC free article] [PubMed] [Google Scholar]
- Levin B., Dobbs R. H., Burgess E. A., Palmer T. Hyperammonaemia. A variant type of deficiency of liver ornithine transcarbamylase. Arch Dis Child. 1969 Apr;44(234):162–169. doi: 10.1136/adc.44.234.162. [DOI] [PMC free article] [PubMed] [Google Scholar]
- Levin B. Hereditary metabolic disorders of the urea cycle. Adv Clin Chem. 1971;14:65–143. doi: 10.1016/s0065-2423(08)60145-6. [DOI] [PubMed] [Google Scholar]
- MYERS D. K., SLATER E. C. The enzymic hydrolysis of adenosine triphosphate by liver mitochondria. I. Activities at different pH values. Biochem J. 1957 Dec;67(4):558–572. doi: 10.1042/bj0670558. [DOI] [PMC free article] [PubMed] [Google Scholar]
- Mallet L. E., Exton J. H., Park C. R. Control of gluconeogenesis from amino acids in the perfused rat liver. J Biol Chem. 1969 Oct 25;244(20):5713–5723. [PubMed] [Google Scholar]
- Marshall M., Cohen P. P. Ornithine transcarbamylase from Streptococcus faecalis and bovine liver. II. Multiple binding sites for carbamyl-P and L-norvaline, correlation with steady state kinetics. J Biol Chem. 1972 Mar 25;247(6):1654–1668. [PubMed] [Google Scholar]
- RATNER S., MORELL H., CARVALHO E. Enzymes of arginine metabolism in brain. Arch Biochem Biophys. 1960 Dec;91:280–289. doi: 10.1016/0003-9861(60)90502-6. [DOI] [PubMed] [Google Scholar]
- RATNER S., PETRACK B. The mechanism of arginine synthesis from citrulline in kidney. J Biol Chem. 1953 Jan;200(1):175–185. [PubMed] [Google Scholar]
- REICHARD H. Ornithine carbamyl transferase activity in human tissue homogenates. J Lab Clin Med. 1960 Aug;56:218–221. [PubMed] [Google Scholar]
- SCHIMKE R. T. Adaptive characteristics of urea cycle enzymes in the rat. J Biol Chem. 1962 Feb;237:459–468. [PubMed] [Google Scholar]
- SCHIMKE R. T. Differential effects of fasting and protein-free diets on levels of urea cycle enzymes in rat liver. J Biol Chem. 1962 Jun;237:1921–1924. [PubMed] [Google Scholar]
- SCHIMKE R. T. Studies on factors affecting the levels of urea cycle enzymes in rat liver. J Biol Chem. 1963 Mar;238:1012–1018. [PubMed] [Google Scholar]
- Schnaitman C., Greenawalt J. W. Enzymatic properties of the inner and outer membranes of rat liver mitochondria. J Cell Biol. 1968 Jul;38(1):158–175. doi: 10.1083/jcb.38.1.158. [DOI] [PMC free article] [PubMed] [Google Scholar]
- Shigesada K., Tatibana M. Role of acetylglutamate in ureotelism. I. Occurrence and biosynthesis of acetylglutamate in mouse and rat tissues. J Biol Chem. 1971 Sep 25;246(18):5588–5595. [PubMed] [Google Scholar]
- Snodgrass P. J. The effects of pH on the kinetics of human liver Ornithine--carbamyl phosphate transferase. Biochemistry. 1968 Sep;7(9):3047–3051. doi: 10.1021/bi00849a004. [DOI] [PubMed] [Google Scholar]
- TAMIR H., RATNER S. A STUDY OF ORNITHINE, CITRULLINE AND ARGININE SYNTHESIS IN GROWING CHICKS. Arch Biochem Biophys. 1963 Aug;102:259–269. doi: 10.1016/0003-9861(63)90179-6. [DOI] [PubMed] [Google Scholar]
- WOLLENBERGER A., RISTAU O., SCHOFFA G. [A simple technic for extremely rapid freezing of large pieces of tissue]. Pflugers Arch Gesamte Physiol Menschen Tiere. 1960;270:399–412. [PubMed] [Google Scholar]
- Williamson D. H., Lund P., Krebs H. A. The redox state of free nicotinamide-adenine dinucleotide in the cytoplasm and mitochondria of rat liver. Biochem J. 1967 May;103(2):514–527. doi: 10.1042/bj1030514. [DOI] [PMC free article] [PubMed] [Google Scholar]