title: Activity of orotate metabolizing enzyme complex and various urea-cycle enzymes in mutant mice with ornithine transcarbamylase deficiency.
abstract: The overall activity of the enzyme complex consisting of orotate phosphoribosyl transferase and orotidine monophosphate decarboxylase, and of various enzymes of the urea cycle, has ben studied in sparse-fur (spf) mutant mice with ornithine transcarbamylase deficiency. The enzyme complex has a lower overall activity, which could be caused by disturbed pyrimidine metabolism due to hyperammonemia. Other enzymes of the urea cycle do not show any significant change.
mesh_terms: D000818:Animals; D051379:Mice; D008817:Mice, Mutant Strains; D009097:Multienzyme Complexes / Q000378:metabolism; D020163:Ornithine Carbamoyltransferase Deficiency Disease; D009962:Orotate Phosphoribosyltransferase / Q000378:metabolism; D009963:Orotic Acid / Q000378:metabolism*; D009964:Orotidine-5'-Phosphate Decarboxylase / Q000378:metabolism; D014508:Urea / Q000378:metabolism*
chemical_list: D009097:Multienzyme Complexes; D009963:Orotic Acid; D014508:Urea; D009962:Orotate Phosphoribosyltransferase; D009964:Orotidine-5'-Phosphate Decarboxylase