Ornithine transcarbamylase (OTC) (also called ornithine carbamoyltransferase) is an enzyme (EC 2.1.3.3) that catalyzes the reaction between carbamoyl phosphate (CP) and ornithine (Orn) to form citrulline (Cit) and phosphate (Pi). There are two classes of OTC: anabolic and catabolic. This article focuses on anabolic OTC. Anabolic OTC facilitates the sixth step in the biosynthesis of the amino acid arginine in prokaryotes.[5] In contrast, mammalian OTC plays an essential role in the urea cycle, the purpose of which is to capture toxic ammonia and transform it into urea, a less toxic nitrogen source, for excretion.
^ abcGRCh38: Ensembl release 89: ENSG00000036473 – Ensembl, May 2017
^ abcGRCm38: Ensembl release 89: ENSMUSG00000031173 – Ensembl, May 2017
^"Human PubMed Reference:". National Center for Biotechnology Information, U.S. National Library of Medicine.
^"Mouse PubMed Reference:". National Center for Biotechnology Information, U.S. National Library of Medicine.
^Cunin R, Glansdorff N, Piérard A, Stalon V (September 1986). "Biosynthesis and metabolism of arginine in bacteria". Microbiological Reviews. 50 (3): 314–52. doi:10.1128/mr.50.3.314-352.1986. PMC 373073. PMID 3534538.
and 24 Related for: Ornithine transcarbamylase information
Ornithinetranscarbamylase (OTC) (also called ornithine carbamoyltransferase) is an enzyme (EC 2.1.3.3) that catalyzes the reaction between carbamoyl...
Ornithinetranscarbamylase deficiency also known as OTC deficiency is the most common urea cycle disorder in humans. Ornithinetranscarbamylase, the defective...
abnormally accumulated in the body in ornithine transcarbamylase deficiency. The radical is ornithyl. L-Ornithine is one of the products of the action of the...
of the enzymes in the urea cycle. The most common example is ornithinetranscarbamylase deficiency, which is inherited in an X-linked fashion. Secondary...
the urea cycle in which it reacts with ornithine (a process catalyzed by the enzyme ornithinetranscarbamylase) to form citrulline. Carbamoyl phosphate...
in a clinical trial for gene therapy. Gelsinger suffered from ornithinetranscarbamylase deficiency, an X-linked genetic disease of the liver, the symptoms...
synthetase I. The second step facilitated by ornithinetranscarbamylase converts carbamoyl phosphate and ornithine into citrulline. After these initial steps...
antidiuretic hormone. Another rare cause of a decreased BUN is ornithinetranscarbamylase deficiency, which is a genetic disorder inherited in an X-linked...
arise secondary to blockage of the urea cycle, particularly in ornithinetranscarbamylase deficiency (OTC deficiency). This can be distinguished from hereditary...
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and on the chest and stomach. A similar condition is linked to OrnithineTranscarbamylase Deficiency in mice. Barr body Bicolor cat Calico cat Deaf white...
defects in the enzymes responsible for the urea cycle, such as ornithinetranscarbamylase, lead to hyperammonemia. Hyperammonemia contributes to the confusion...
mimics a classic urea cycle defect, such as ornithinetranscarbamylase deficiency, as the block in ornithine metabolism leads to secondary dysfunction of...
Just as in the urea cycle, in its synthesis, ornithine is replaced by lysine. Ornithinetranscarbamylase is the main enzyme for homoarginine synthesis...
1s (GSD1a). DTX301 - in clinical development for treatment of ornithinetranscarbamylase (OTC) deficiency. DTX201/BAY 2599023 - in clinical development...
Gelsinger in a trial of an adenovirus-vectored treatment for ornithinetranscarbamylase deficiency due to a systemic inflammatory reaction led to a temporary...
due to a metabolic disorder, such as a urea cycle disorder. In ornithinetranscarbamylase deficiency, an X-linked inherited and the most common urea cycle...
deficiences in the enzymes carbamoyl phosphate synthetase I, ornithinetranscarbamylase, or argininosuccinic acid synthetase. Uncontrolled, this causes...
identify key amino acids. Proximal urea cycle defects, such as ornithinetranscarbamylase deficiency and carbamoyl phosphate synthetase deficiency are not...
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