May 1, 1976

Abnormal ornithine carbamoyltransferase in mice having the sparse-fur mutation

Proceedings of the National Academy of Sciences of the United States of America
R DeMarsLiane B Russell


Mice with the X-chromosomal sparse-fur (spf) mutation frequently have urinary bladder stones composed mostly of orotic acid, which was identified by the following criteria: ultraviolet and infrared absorption, spectra, chromatographic behavior, melting point, and reactivity in a specific color test. This clue led to the discovery that spf-bearing mice have an abnormal form of liver ornithine carbamoyltransferase (carbamoylphosphate:L-ornithine carbamoyltransferase, EC Normal ornithine carbamoyltransferase has maximum activity at pH 7.6-8.0 and 80% of maximum activity at pH 10.0.

  • References75
  • Citations8


  • References75
  • Citations8


Mentioned in this Paper

Urinary Bladder Calculi (Disorder)
Sex Chromosomes
Ornithine Monoacetate, (L)-Isomer
Orotic Acid
Color Test
Ornithine Carbamoyltransferase Deficiency
Zinc Orotate

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