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生化/生理作用
Mutations in ornithine transcarbamylase cause Ornithine transcarbamylase deficiency which prevents the clearance of the byproduct ammonia leading to hyperammonemia.
单位定义
在 pH 8.5 和 37°C 下，一单位本品每分钟可由鸟氨酸和氨基甲酰磷酸生成 1.0μmol 瓜氨酸。
外形
含 Tris 缓冲盐的冻干粉
储存分类代码
11 - Combustible Solids
WGK
WGK 3
闪点(°F)
Not applicable
闪点(°C)
Not applicable
Jon Jerlström-Hultqvist et al.
Eukaryotic cell, 11(7), 864-873 (2012-05-23)
In recent years, proteomics has come of age with the development of efficient tools for purification, identification, and characterization of gene products predicted by genome projects. The intestinal protozoan Giardia intestinalis can be transfected, but there is only a limited
Wataru Takagi et al.
Comparative biochemistry and physiology. Part B, Biochemistry & molecular biology, 161(4), 331-340 (2012-01-10)
Cartilaginous fish comprise two subclasses, the Holocephali (chimaeras) and Elasmobranchii (sharks, skates and rays). Little is known about osmoregulatory mechanisms in holocephalan fishes except that they conduct urea-based osmoregulation, as in elasmobranchs. In the present study, we examined the ornithine
Luis Mariano Polo et al.
PloS one, 7(2), e31528-e31528 (2012-03-01)
Transcarbamylases reversibly transfer a carbamyl group from carbamylphosphate (CP) to an amine. Although aspartate transcarbamylase and ornithine transcarbamylase (OTC) are well characterized, little was known about putrescine transcarbamylase (PTC), the enzyme that generates CP for ATP production in the fermentative
Taiichi Wakiya et al.
Molecular genetics and metabolism, 105(3), 404-407 (2012-01-24)
There are no objective and concrete guidelines for the management of Ornithine transcarbamylase deficiency (OTCD). Based on previous findings, we hypothesized that patients with OTCD have a low Ornithine transcarbamylase (OTC) activity in the liver, and therefore it would be
Jing Wang et al.
Molecular genetics and metabolism, 106(2), 221-230 (2012-04-13)
Oligonucleotide array-based comparative genomic hybridization (aCGH) targeted to coding exons of genes of interest has been proven to be a valuable diagnostic tool to complement with Sanger sequencing for the detection of large deletions/duplications. We have developed a custom designed
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