- Tel: 858.663.9055
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Email: info@nsjbio.com
- Tel: 858.663.9055
- Email: info@nsjbio.com
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Ornithine Transcarbamylase Antibody recognizes ornithine transcarbamylase (OTC), a mitochondrial enzyme that catalyzes a key step in the urea cycle by transferring a carbamoyl group from carbamoyl phosphate to ornithine, generating citrulline. This reaction is essential for the detoxification of ammonia produced during amino acid metabolism and represents one of the central pathways maintaining whole-body nitrogen balance. Because OTC activity is largely restricted to hepatocytes and, to a lesser extent, intestinal epithelial cells, it is a critical marker for studies of hepatic metabolism and mitochondrial function. NSJ Bioreagents provides Ornithine Transcarbamylase Antibody for investigations of nitrogen metabolism, liver biology, and mitochondrial enzymes.
OTC is localized within the mitochondrial matrix, where it functions in concert with the other enzymes of the urea cycle to convert toxic ammonia into urea for renal excretion. Proper regulation of this pathway is essential for maintaining normal neurological function and metabolic homeostasis. Alterations in OTC expression or activity can impair ammonia clearance, resulting in hyperammonemia and widespread metabolic consequences. Consequently, an Ornithine Transcarbamylase Antibody is valuable for studies examining mitochondrial metabolism, hepatic physiology, and the regulation of nitrogen disposal.
Inherited deficiency of OTC is the most common urea cycle disorder and is caused by pathogenic variants in the X-linked OTC gene. The resulting enzyme deficiency can produce a broad clinical spectrum ranging from severe neonatal hyperammonemia to later-onset metabolic disease. Beyond inherited disorders, OTC expression has also been investigated in liver development, hepatocellular function, metabolic adaptation, and experimental models of liver injury and mitochondrial dysfunction.
An Ornithine Transcarbamylase Antibody is a valuable research tool for investigating urea cycle biology, mitochondrial metabolism, ammonia detoxification, hepatic function, nitrogen homeostasis, and Ornithine Transcarbamylase expression.
Explore our Metabolism Antibodies page to discover additional antibodies for investigating metabolic enzymes, mitochondrial proteins, amino acid metabolism, and cellular energy pathways.
The stated application concentrations are suggested starting points. Titration of the Ornithine transcarbamylase antibody may be required due to differences in protocols and secondary/substrate sensitivity.
A portion of amino acids 71-98 from the human protein was used as the immunogen for the Ornithine transcarbamylase antibody.
Aliquot the Ornithine transcarbamylase antibody and store frozen at -20oC or colder. Avoid repeated freeze-thaw cycles.
Ornithine Transcarbamylase antibody, OTC antibody, OTCase antibody, Ornithine Carbamoyltransferase antibody
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