Ornithine carbamoyltransferase, mitochondrial; Ornithine transcarbamylase; OTCase; OTC; 2.1.3.3

For Research Use Only. Not for use in diagnostic procedures.

Store at 4 degree C

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Small volumes of OTC elisa kit vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

MBS9427330 is a ready-to-use microwell, strip-or-full plate ELISA (enzyme-linked immunosorbent assay) Kit for analyzing the presence of the Ornithine carbamoyltransferase (OTC) ELISA Kit target analytes in biological samples. The concentration gradients of the kit standards or positive controls render a theoretical kit detection range in biological research samples containing OTC. The ELISA analytical biochemical technique of the MBS9427330 kit is based on OTC antibody-OTC antigen interactions (immunosorbency) and an HRP colorimetric detection system to detect OTC antigen targets in samples. The ELISA Kit is designed to detect native, not recombinant, OTC. Appropriate sample types may include undiluted body fluids and/or tissue homogenates, secretions. Quality control assays assessing reproducibility identified the intra-assay CV (%) and inter-assay CV(%).

39,935 Da

ornithine carbamoyltransferase

ornithine carbamoyltransferase, mitochondrial

Ornithine carbamoyltransferase, mitochondrial

OTCase??[Similar Products]

This nuclear gene encodes a mitochondrial matrix enzyme. Missense, nonsense, and frameshift mutations in this enzyme lead to ornithine transcarbamylase deficiency, which causes hyperammonemia. Since the gene for this enzyme maps close to that for Duchenne muscular dystrophy, it may play a role in that disease also. [provided by RefSeq, Jul 2008]

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: Amino Acid Metabolism - arginine and proline; EC 2.1.3.3; Mitochondrial; Transferase

Chromosomal Location of Human Ortholog: Xp11.4

Cellular Component: mitochondrial matrix; mitochondrion

Molecular Function: ornithine carbamoyltransferase activity

Biological Process: arginine biosynthetic process via ornithine; citrulline biosynthetic process; ornithine catabolic process; urea cycle

Disease: Ornithine Transcarbamylase Deficiency, Hyperammonemia Due To

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