Ornithine transcarbamylase; OTCase

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

E Coli or Yeast or Baculovirus or Mammalian Cell

Greater than 90% as determined by SDS-PAGE. (lot specific)

Liquid containing glycerol

This protein contains an N-terminal tag and may also contain a C-terminal tag. Tag types are determined by various factors including tag-protein stability, please inquire for tag information.

Sterile filter available upon request.

Low endotoxin available upon request.

Store at -20 degree C, for extended storage, conserve at -20 degree C or -80 degree C.

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

Small volumes of Otc 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.

(Note: Representative image, actual molecular weight may vary depending on Tag type and expression host)

52.1kD

ornithine transcarbamylase

ornithine carbamoyltransferase, mitochondrial

Ornithine carbamoyltransferase, mitochondrial

OTCase??[Similar Products]

OTC_MOUSE

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

Cellular Component: cytoplasm; mitochondrial inner membrane; mitochondrion

Molecular Function: amino acid binding; carboxyl- or carbamoyltransferase activity; ornithine carbamoyltransferase activity; phosphate binding; phospholipid binding; transferase activity

Biological Process: amino acid biosynthetic process; amino acid metabolic process; anion homeostasis; arginine biosynthetic process; arginine biosynthetic process via ornithine; citrulline biosynthetic process; ornithine catabolic process; ornithine metabolic process; urea cycle

The molecular basis of the sparse fur mouse mutation.Veres G., Gibbs R.A., Scherer S.E., Caskey C.T.Science 237:415-417(1987) The genetic structure of mouse ornithine transcarbamylase.Scherer S.E., Veres G., Caskey C.T.Nucleic Acids Res. 16:1593-1601(1988) The 5' flanking region of the ornithine transcarbamylase gene contains DNA sequences regulating tissue-specific expression.Veres G., Craigen W.J., Caskey C.T.J. Biol. Chem. 261:7588-7591(1986) SIRT5-mediated lysine desuccinylation impacts diverse metabolic pathways.Park J., Chen Y., Tishkoff D.X., Peng C., Tan M., Dai L., Xie Z., Zhang Y., Zwaans B.M., Skinner M.E., Lombard D.B., Zhao Y.Mol. Cell 50:919-930(2013) Label-free quantitative proteomics of the lysine acetylome in mitochondria identifies substrates of SIRT3 in metabolic pathways.Rardin M.J., Newman J.C., Held J.M., Cusack M.P., Sorensen D.J., Li B., Schilling B., Mooney S.D., Kahn C.R., Verdin E., Gibson B.W.Proc. Natl. Acad. Sci. U.S.A. 110:6601-6606(2013)

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