Subcellular location of glutamine synthetase and urea cycle enzymes in liver of spiny dogfish (Squalus acanthias).
Glutamine synthetase and glutamine- and acetylglutamate-dependent carbamoyl-phosphate synthetase, both of which are present in high concentrations in liver of urea-retaining elasmobranchs, have been found to be located exclusively in the mitochondria in liver from the representative elasmobranch Squalus acanthias. This observation is consistent with the view that the function of this unique carbamoyl-phosphate synthetase is related to urea synthesis, and that the initial nitrogen-donating substrate for urea synthesis in these species is glutamine rather than ammonia. The urea cycle enzymes, ornithine carbamoyltransferase and arginase, are also located in the mitochondria, whereas argininosuccinate synthetase and argininosuccinate lyase are located in the cytosol. Glutamine synthetase and arginase are mitochondrial enzymes in uricotelic species, but are normally found in the cytoplasm in ureotelic species. the properties of the elasmobranch arginase, however, are characteristic of arginases from ureotelic species (e.g. the Km for arginine is 1.2 mM, and the enzyme has an Mr congruent to 100,000).
D000818:Animals; D002223:Carbamoyl-Phosphate Synthase (Glutamine-Hydrolyzing) / Q000378:metabolism*; D019731:Carbon-Nitrogen Ligases; D002458:Cell Fractionation; D002467:Cell Nucleus / Q000201:enzymology / Q000648:ultrastructure; D004284:Dogfish; D005974:Glutamate-Ammonia Ligase / Q000378:metabolism*; D008025:Ligases / Q000378:metabolism*; D008099:Liver / Q000201:enzymology*; D008854:Microscopy, Electron; D008930:Mitochondria, Liver / Q000201:enzymology / Q000648:ultrastructure; D013347:Subcellular Fractions / Q000201:enzymology; D014508:Urea / Q000378:metabolism*
D014508:Urea; D008025:Ligases; D019731:Carbon-Nitrogen Ligases; D005974:Glutamate-Ammonia Ligase; C024233:carbamoyl-phosphate synthetase (N-acetylglutamate); D002223:Carbamoyl-Phosphate Synthase (Glutamine-Hydrolyzing)