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

Chromosome: X; NC_000023.10 (38211736..38280703). Location: Xp21.1

E Coli

> 95%

Supplied as lyophilized form in PBS,pH7.4, containing 5% sucrose, 0.01% sarcosyl.

Avoid repeated freeze/thaw cycles. Store at 2-8 degree C for one month. Aliquot and store at -80 degree C for 12 months.
Stability Test: The thermal stability is described by the loss rate of the targetprotein. The loss rate was determined by accelerated thermal degradation test,that is, incubate the protein at 37 degree C for 48h, and no obvious degradation andprecipitation were observed. (Referring from China Biological Products Standard,which was calculated by the Arrhenius equation.) The loss of this protein is lessthan 5% within the expiration date under appropriate storage condition.

Manufactured in an ISO 9001:2008 and ISO 13485:2003 Certified Laboratory.

Manufactured in a lab with traceable raw materials. Bulk orders can typically be prepared to the customera??s specifications, please inquire.

Small volumes of OCT recombinant protein 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.

About the Marker: Effective Size Range: 10kDa to 70kDa.
Protein bands: 10kDa, 14kDa, 18kDa, 22kDa, 26kDa, 33kDa, 44kDa and70kDa.
Double intensity bands: The 26kDa, 18kDa, 10kDa bands are at doubleintensity to make location and size approximation of proteins of interestquick and easy.
Ready-to-use: No need to heat, dilute or add reducing agents before use.

SDS-PAGE, Western Blot (WB), ELISA (EIA), Immunoprecipitation (IP)

33.2kDa

ornithine carbamoyltransferase

ornithine carbamoyltransferase, mitochondrial; OTCase; ornithine transcarbamylase

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

Chromosomal Location of Human Ortholog: Xp21.1

Cellular Component: mitochondrion; mitochondrial matrix; mitochondrial inner membrane

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

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

Disease: Ornithine Transcarbamylase Deficiency, Hyperammonemia Due To

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