Recombinant Mouse Ornithine carbamoyltransferase, mitochondrial | MyBioSource MBS960037 product information

product summary

company name :

MyBioSource

product type :

protein

product name :

Recombinant Mouse Ornithine carbamoyltransferase, mitochondrial

catalog :

MBS960037

quantity :

0.05 mg (E-Coli)

price :

180 USD

more info or order :

MyBioSource product webpage

product information

catalog number :

MBS960037

products type :

Recombinant Protein

products full name :

Recombinant Mouse Ornithine carbamoyltransferase, mitochondrial

products short name :

Ornithine carbamoyltransferase

products name syn :

Ornithine transcarbamylase; OTCase

other names :

ornithine carbamoyltransferase, mitochondrial; Ornithine carbamoyltransferase, mitochondrial; ornithine carbamoyltransferase, mitochondrial; ornithine transcarbamylase; Ornithine transcarbamylase; OTCase

products gene name :

Otc

other gene names :

Otc; Otc; Sf; spf; AI265390; OTCase

uniprot entry name :

OTC_MOUSE

host :

E Coli or Yeast or Baculovirus or Mammalian Cell

sequence positions :

33-354; Mature full length protein

sequence length :

354

sequence :

RLSTETGFALLGGHPSFLTTQDIHLGVNESLTDTARVLSSMTDAVLARVYKQSDLDTLAKE ASIPIVNGLSDLYHPIQILADYLTLQEHYGSLKGLTLSWIGDGNNILHSIMMSAAKFGMHLQ AATPKGYEPDPNIVKLAEQYAKENGTKLSMTNDPLEAARGGNVLITDTWISMGQEDEKK KRLQAFQGYQVTMKTAKVAASDWTFLHCLPRKPEEVDDEVFYSPRSLVFPEAENRKW TIMAVMVSLLTDYSPVLQKPKF
SQVQLKGRDLLTLKNFTGEEIQYMLWLSADLKFRIKQKG
EYLPLLQGKSLGMIFEKRSTRT

purity :

Greater than 90% as determined by SDS-PAGE.

form :

Liquid containing glycerol; lyophilization may be available upon request.

storage stability :

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

products references :

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)

ncbi gi num :

6679184

ncbi acc num :

NP_032795.1

ncbi gb acc num :

NM_008769.4

uniprot acc num :

P11725

ncbi mol weight :

52.1kD

ncbi pathways :

Amino Acid Metabolism Pathway (198416); Arginine Biosynthesis Pathway (83143); Arginine Biosynthesis Pathway (306); Biosynthesis Of Amino Acids Pathway (790952); Biosynthesis Of Amino Acids Pathway (795174); Metabolic Pathways (132962); Metabolism Pathway (1324226); Metabolism Of Amino Acids And Derivatives Pathway (1324419); Metabolism Of Polyamines Pathway (1324430); Urea Cycle Pathway (421751)

uniprot summary :

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

size1 :

0.05 mg (E-Coli)

price1 :

180 USD

size2 :

0.2 mg (E-Coli)

price2 :

490

size3 :

0.5 mg (E-Coli)

price3 :

715

size4 :

0.05 mg (Baculovirus)

price4 :

950

size5 :

1 mg (E-Coli)

price5 :

1170

more info or order :

MyBioSource product webpage