Assays for aspartate and ornithine transcarbamylase by means of the pH-stat

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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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Increase of ornithine transcarbamylase protein in sparse-fur mice with ornithine transcarbamylase deficiency.

Ornithine transcarbamylase (OTCase, EC 2.1.3.3) a mitochondrial enzyme of the urea cycle catalyses the formation of citrulline from ornithine and carbamoyl phosphate. It has been purified from liver in 3 mammalian species: bovine [ 11, human [2] and rat [3,4] but not from mouse. We decided to purify mouse OTCase because the mouse is the only mammalian species in which two strains with an OTCase...

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ژورنال

عنوان ژورنال: Fungal Genetics Reports

سال: 1965

ISSN: 1941-4765

DOI: 10.4148/1941-4765.2126