Rat Ornithine carbamoyltransferase, mitochondrial ELISA Kit | Otc elisa kit
Rat Ornithine carbamoyltransferase, mitochondrial ELISA Kit
NCBI and Uniprot Product Information
NCBI Description
catalyzes the conversion of L-ornithine to L-citrulline [RGD, Feb 2006]
Uniprot Description
OTC: Defects in OTC are the cause of ornithine carbamoyltransferase deficiency (OTCD). OTCD is an X- linked disorder of the urea cycle which causes a form of hyperammonemia. Mutations with no residual enzyme activity are always expressed in hemizygote males by a very severe neonatal hyperammonemic coma that generally proves to be fatal. Heterozygous females are either asymptomatic or express orotic aciduria spontaneously or after protein intake. The disorder is treatable with supplemental dietary arginine and low protein diet. The arbitrary classification of patients into the 'neonatal' group (clinical hyperammonemia in the first few days of life) and 'late' onset (clinical presentation after the neonatal period) has been used to differentiate severe from mild forms. Belongs to the ATCase/OTCase family.
Protein type: Lipid-binding; Transferase; Mitochondrial; EC 2.1.3.3; Amino Acid Metabolism - arginine and proline
Cellular Component: cytoplasm; mitochondrial inner membrane; mitochondrial matrix; mitochondrion
Molecular Function: amino acid binding; ornithine carbamoyltransferase activity; phosphate binding; phospholipid binding
Biological Process: anion homeostasis; arginine biosynthetic process via ornithine; citrulline biosynthetic process; liver development; midgut development; ornithine catabolic process; ornithine metabolic process; response to drug; response to insulin stimulus; response to nutrient levels; response to zinc ion; urea cycle
Research Articles on Otc
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Product Notes
The Rat Otc otc (Catalog #AAA2880392) is an ELISA Kit and is intended for research purposes only. The product is available for immediate purchase. The AAA2880392 ELISA Kit recognizes Rat Otc. It is sometimes possible for the material contained within the vial of "Ornithine carbamoyltransferase, mitochondrial, ELISA Kit" to become dispersed throughout the inside of the vial, particularly around the seal of said vial, during shipment and storage. We always suggest centrifuging these vials to consolidate all of the liquid away from the lid and to the bottom of the vial prior to opening. Please be advised that certain products may require dry ice for shipping and that, if this is the case, an additional dry ice fee may also be required.Precautions
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