Activity of orotate metabolizing enzyme complex and various urea-cycle enzymes in mutant mice with ornithine transcarbamylase deficiency.
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.
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*
D009097:Multienzyme Complexes; D009963:Orotic Acid; D014508:Urea; D009962:Orotate Phosphoribosyltransferase; D009964:Orotidine-5'-Phosphate Decarboxylase