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  • 标题:Abnormal ornithine carbamoyltransferase in mice having the sparse-fur mutation
  • 本地全文:下载
  • 作者:R DeMars ; S L LeVan ; B L Trend
  • 期刊名称:Proceedings of the National Academy of Sciences
  • 印刷版ISSN:0027-8424
  • 电子版ISSN:1091-6490
  • 出版年度:1976
  • 卷号:73
  • 期号:5
  • 页码:1693-1697
  • DOI:10.1073/pnas.73.5.1693
  • 语种:English
  • 出版社:The National Academy of Sciences of the United States of America
  • 摘要: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 2.1.3.3 ). Normal ornithine carbamoyltransferase has maximum activity at pH 7.6-8.0 and 80% of maximum activity at pH 10.0.
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