OTC/Ornithine carbamoyltransferase; mitochondrial/Ornithine transcarbamylase/OTCase

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

Store at 4 degree C if kit is to be used within 1 week. Stable for 6 months (if micro ELISA Plate, Lyophilized Standard and Concentrated Biotinylated Detection Protein stored at-20 degree C. Other components at 2-8 degree C). Stable for 12 months (if the entire kit is stored at-20 degree C).

Manufactured in an ISO 13485:2003 and EN ISO 13485:2012 Certified Laboratory.

Select online data sheet information is drawn from bioinformatics databases, occasionally resulting in ambiguous or non-relevant product information. It is the responsibility of the customer to review, verify, and evaluate the information to make sure it matches their requirements before purchasing the kit. Our ELISA Kit assays are dynamic research tools and sometimes they may be updated and improved. If the format of this assay is important to you then please request the current manual or contact our technical support team with a presales inquiry before placing an order. We will confirm the current details of the assay. We cannot guarantee the sample manual posted online is the most current manual.

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.

MBS764060 is a ready-to-use microwell, strip plate ELISA (enzyme-linked immunosorbent assay) Kit for analyzing the presence of the Ornithine carbamoyltransferase, mitochondrial (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 MBS764060 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(%).

Principle of the Assay||This kit was based on sandwich enzyme-linked immune-sorbent assay technology. Anti-Human OTCantibody was pre-coated onto 96-well plates. And the biotin conjugated anti-Human OTC antibody was used as detection antibodies. The standards, test samples and biotin conjugated detection antibody were added to the wells subsequently, and wash with wash buffer. HRPStreptavidin was added and unbound conjugates were washed away with wash buffer. TMB substrates were used to visualize HRP enzymatic reaction. TMB was catalyzed by HRP to produce a blue color product that changed into yellow after adding acidic stop solution. The density of yellow is proportional to the Human OTC amount of sample captured in plate. Read the O.D. absorbance at 450nm in a microplate reader, and then the concentration ofHuman OTCcan be calculated.

39,935 Da

ornithine carbamoyltransferase

ornithine carbamoyltransferase, mitochondrial

Ornithine carbamoyltransferase, mitochondrial

OTCase??[Similar Products]

OTC_HUMAN

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: Xp21.1

Cellular Component: mitochondrial inner membrane; mitochondrial matrix; mitochondrion

Molecular Function: amino acid binding; ornithine carbamoyltransferase activity; phosphate binding; phospholipid binding

Biological Process: arginine biosynthetic process via ornithine; citrulline biosynthetic process; liver development; midgut development; ornithine catabolic process; polyamine metabolic process; response to drug; response to insulin stimulus; response to zinc ion; urea cycle

Disease: Ornithine Transcarbamylase Deficiency, Hyperammonemia Due To

All of MyBioSource's Products are for scientific laboratory research purposes and are not for diagnostic, therapeutics, prophylactic or in vivo use. Through your purchase, you expressly represent and warrant to MyBioSource that you will properly test and use any Products purchased from MyBioSource in accordance with industry standards. MyBioSource and its authorized distributors reserve the right to refuse to process any order where we reasonably believe that the intended use will fall outside of our acceptable guidelines.

While every efforts were made to ensure the accuracy of the information provided in this datasheet, MyBioSource will not be liable for any omissions or errors contained herein. MyBioSource reserves the right to make changes to this datasheet at any time without prior notice.

It is the responsibility of the customer to report product performance issues to MyBioSource within 30 days of receipt of the product. Please visit our Terms & Conditions page for more information.