Ornithine Transcarbamylase from Streptococcus faecalis and Bovine Liver

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Ornithine Transcarbamylase Deficiency

Accumulation of ammonia in body tissues most commonly occurs in hepatic failure. However, it may also result from a specific deficiency of an enzyme of the urea cycle (Fig., 1). Severe hyperammonaemia has been reported with deficiencies of carbamyl phosphate synthetase (Freeman et al., 1964) and ornithine transcarbamylase (Russell et al., 1962; Levin and Russell, 1967). Some increase in blood a...

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Antepartum Ornithine Transcarbamylase Deficiency

Ornithine transcarbamylase deficiency (OTCD) is the most common type urea cycle enzyme deficiencies. This syndrome results from a deficiency of the mitochondrial enzyme ornithine transcarbamylase, which catalyzes the conversion of ornithine and carbamoyl phosphate to citrullin. Our case was a 28-year-old female diagnosed with OTCD following neurocognitive deficit during her first pregnancy. Alt...

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[Ornithine transcarbamylase deficiency].

Accumulation of ammonia in body tissues most commonly occurs in hepatic failure. However, it may also result from a specific deficiency of an enzyme of the urea cycle (Fig., 1). Severe hyperammonaemia has been reported with deficiencies of carbamyl phosphate synthetase (Freeman et al., 1964) and ornithine transcarbamylase (Russell et al., 1962; Levin and Russell, 1967). Some increase in blood a...

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Orthotopic liver transplantation for ornithine transcarbamylase deficiency with hyperammonemic encephalopathy.

Ornithine transcarbamylase (OTC) deficiency is an X chromosome-linked disorder causing hyperammonemic encephalopathy with a very poor prognosis. We describe here two patients with OTC deficiency, one a late on-set female patient (case 1) and the other a neonatal-onset male patient (case 2), who were successfully treated with orthotopic liver transplantation (OLTx). The OTC activity in the excis...

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Hyperammonaemia. A variant type of deficiency of liver ornithine transcarbamylase.

The specific syndrome arising from an absent or low hepatic ornithine transcarbamylase activity has been termed hyperammonaemia (Russell et al., 1962; Levin and Russell, 1967; Levin, 1968). In the previous communication (Levin et al., 1969) 2 cases occurring in mother and child are described. In this article, we record an infant who during the course of an investigation for the cause of his vom...

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ژورنال

عنوان ژورنال: Journal of Biological Chemistry

سال: 1972

ISSN: 0021-9258

DOI: 10.1016/s0021-9258(19)45528-2