Ornithine Carbamoyl Transferase (OCT)
OCTD; OTCase; Ornithine Transcarbamylase; Ornithine Carbamoyltransferase
Ornithine transcarbamoylase (OTC) (also called ornithine carbamoyltransferase) is an enzyme that catalyzes the reaction between carbamoyl phosphate (CP) and ornithine (Orn) to form citrulline (Cit) and phosphate (Pi). In plants and microbes OTC is involved in arginine (Arg) biosynthesis, whereas in mammals it is located in the mitochondria and is part of the urea cycle.OTC is a trimer.
The monomer unit has a CP-binding domain and an amino acid-binding domain. Each of the two discrete substrate-binding domains (SBDs) have an alpha/beta; topology with a central beta-pleated sheet embedded in flanking alpha-helices.The active sites are located at the interface between the protein monomers.
Organism species: Homo sapiens (Human)
PRODUCT TYPE | CATALOG NO. | PRODUCT NAME | APPLICATIONS |
---|---|---|---|
Proteins | RPV342Hu01 | Recombinant Ornithine Carbamoyl Transferase (OCT) | Positive Control; Immunogen; SDS-PAGE; WB. |
Antibodies | MAV342Hu01 | Monoclonal Antibody to Ornithine Carbamoyl Transferase (OCT) | WB; IHC; ICC; IP. |
PAV342Hu01 | Polyclonal Antibody to Ornithine Carbamoyl Transferase (OCT) | WB; IHC; ICC; IF. | |
Assay Kits | CLV342Hu01 | CLIA Kit for Ornithine Carbamoyl Transferase (OCT) | CLIA assay for Antigen Detection |
ELV342Hu01 | ELISA Kit for Ornithine Carbamoyl Transferase (OCT) | Enzyme-linked immunosorbent assay for Antigen Detection |
Organism species: Mus musculus (Mouse)
PRODUCT TYPE | CATALOG NO. | PRODUCT NAME | APPLICATIONS |
---|---|---|---|
Proteins | RPV342Mu01 | Recombinant Ornithine Carbamoyl Transferase (OCT) | Positive Control; Immunogen; SDS-PAGE; WB. |
Antibodies | MAV342Mu01 | Monoclonal Antibody to Ornithine Carbamoyl Transferase (OCT) | WB; IHC; ICC; IP. |
PAV342Mu01 | Polyclonal Antibody to Ornithine Carbamoyl Transferase (OCT) | WB; IHC; ICC; IF. | |
Assay Kits | CLV342Mu01 | CLIA Kit for Ornithine Carbamoyl Transferase (OCT) | CLIA assay for Antigen Detection |
ELV342Mu01 | ELISA Kit for Ornithine Carbamoyl Transferase (OCT) | Enzyme-linked immunosorbent assay for Antigen Detection |
Organism species: Rattus norvegicus (Rat)
PRODUCT TYPE | CATALOG NO. | PRODUCT NAME | APPLICATIONS |
---|---|---|---|
Proteins | RPV342Ra01 | Recombinant Ornithine Carbamoyl Transferase (OCT) | Positive Control; Immunogen; SDS-PAGE; WB. |
Antibodies | MAV342Ra01 | Monoclonal Antibody to Ornithine Carbamoyl Transferase (OCT) | WB; IHC; ICC; IP. |
PAV342Ra01 | Polyclonal Antibody to Ornithine Carbamoyl Transferase (OCT) | WB; IHC; ICC; IF. | |
Assay Kits | CLV342Ra01 | CLIA Kit for Ornithine Carbamoyl Transferase (OCT) | CLIA assay for Antigen Detection |
ELV342Ra01 | ELISA Kit for Ornithine Carbamoyl Transferase (OCT) | Enzyme-linked immunosorbent assay for Antigen Detection |
Organism species: Canis familiaris; Canine (Dog)
PRODUCT TYPE | CATALOG NO. | PRODUCT NAME | APPLICATIONS |
---|---|---|---|
Proteins | RPV342Ca01 | Recombinant Ornithine Carbamoyl Transferase (OCT) | Positive Control; Immunogen; SDS-PAGE; WB. |
Antibodies | MAV342Ca01 | Monoclonal Antibody to Ornithine Carbamoyl Transferase (OCT) | WB; IHC; ICC; IP. |
PAV342Ca01 | Polyclonal Antibody to Ornithine Carbamoyl Transferase (OCT) | WB; IHC; ICC; IF. | |
Assay Kits | CLV342Ca01 | CLIA Kit for Ornithine Carbamoyl Transferase (OCT) | CLIA assay for Antigen Detection |
ELV342Ca01 | ELISA Kit for Ornithine Carbamoyl Transferase (OCT) | Enzyme-linked immunosorbent assay for Antigen Detection |
Organism species: Sus scrofa; Porcine (Pig)
PRODUCT TYPE | CATALOG NO. | PRODUCT NAME | APPLICATIONS |
---|---|---|---|
Proteins | RPV342Po01 | Recombinant Ornithine Carbamoyl Transferase (OCT) | Positive Control; Immunogen; SDS-PAGE; WB. |
Antibodies | MAV342Po01 | Monoclonal Antibody to Ornithine Carbamoyl Transferase (OCT) | WB; IHC; ICC; IP. |
PAV342Po01 | Polyclonal Antibody to Ornithine Carbamoyl Transferase (OCT) | WB; IHC; ICC; IF. | |
Assay Kits | CLV342Po01 | CLIA Kit for Ornithine Carbamoyl Transferase (OCT) | CLIA assay for Antigen Detection |
ELV342Po01 | ELISA Kit for Ornithine Carbamoyl Transferase (OCT) | Enzyme-linked immunosorbent assay for Antigen Detection |
Organism species: Bos taurus; Bovine (Cattle)
PRODUCT TYPE | CATALOG NO. | PRODUCT NAME | APPLICATIONS |
---|---|---|---|
Proteins | RPV342Bo01 | Recombinant Ornithine Carbamoyl Transferase (OCT) | Positive Control; Immunogen; SDS-PAGE; WB. |
Antibodies | MAV342Bo01 | Monoclonal Antibody to Ornithine Carbamoyl Transferase (OCT) | WB; IHC; ICC; IP. |
PAV342Bo01 | Polyclonal Antibody to Ornithine Carbamoyl Transferase (OCT) | WB; IHC; ICC; IF. | |
Assay Kits | CLV342Bo01 | CLIA Kit for Ornithine Carbamoyl Transferase (OCT) | CLIA assay for Antigen Detection |
ELV342Bo01 | ELISA Kit for Ornithine Carbamoyl Transferase (OCT) | Enzyme-linked immunosorbent assay for Antigen Detection |
Organism species: Oryctolagus cuniculus (Rabbit)
PRODUCT TYPE | CATALOG NO. | PRODUCT NAME | APPLICATIONS |
---|---|---|---|
Proteins | RPV342Rb01 | Recombinant Ornithine Carbamoyl Transferase (OCT) | Positive Control; Immunogen; SDS-PAGE; WB. |
Antibodies | MAV342Rb01 | Monoclonal Antibody to Ornithine Carbamoyl Transferase (OCT) | WB; IHC; ICC; IP. |
PAV342Rb01 | Polyclonal Antibody to Ornithine Carbamoyl Transferase (OCT) | WB; IHC; ICC; IF. | |
Assay Kits | CLV342Rb01 | CLIA Kit for Ornithine Carbamoyl Transferase (OCT) | CLIA assay for Antigen Detection |
ELV342Rb01 | ELISA Kit for Ornithine Carbamoyl Transferase (OCT) | Enzyme-linked immunosorbent assay for Antigen Detection |
Organism species: Rhesus monkey (Simian)
PRODUCT TYPE | CATALOG NO. | PRODUCT NAME | APPLICATIONS |
---|---|---|---|
Proteins | RPV342Mo01 | Recombinant Ornithine Carbamoyl Transferase (OCT) | Positive Control; Immunogen; SDS-PAGE; WB. |
Antibodies | MAV342Mo01 | Monoclonal Antibody to Ornithine Carbamoyl Transferase (OCT) | WB; IHC; ICC; IP. |
PAV342Mo01 | Polyclonal Antibody to Ornithine Carbamoyl Transferase (OCT) | WB; IHC; ICC; IF. | |
Assay Kits | CLV342Mo01 | CLIA Kit for Ornithine Carbamoyl Transferase (OCT) | CLIA assay for Antigen Detection |
ELV342Mo01 | ELISA Kit for Ornithine Carbamoyl Transferase (OCT) | Enzyme-linked immunosorbent assay for Antigen Detection |
Reference
"Structure and expression of a complementary DNA for the nuclear coded precursor of human mitochondrial ornithine transcarbamylase."
Science 224:1068-1074(1984) [PubMed] [Europe PMC] [Abstract]
"Structure of the human ornithine transcarbamylase gene."
J. Biochem. 103:302-308(1988) [PubMed] [Europe PMC] [Abstract]
"Complete sequencing and characterization of 21,243 full-length human cDNAs."
Nat. Genet. 36:40-45(2004) [PubMed] [Europe PMC] [Abstract]
"The DNA sequence of the human X chromosome."
Nature 434:325-337(2005) [PubMed] [Europe PMC] [Abstract]
"The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC)."
Genome Res. 14:2121-2127(2004) [PubMed] [Europe PMC] [Abstract]
"Arginine in the leader peptide is required for both import and proteolytic cleavage of a mitochondrial precursor."
Proc. Natl. Acad. Sci. U.S.A. 82:4930-4933(1985) [PubMed] [Europe PMC] [Abstract]
"Isolation and characterization of the human ornithine transcarbamylase gene: structure of the 5'-end region."
J. Biochem. 100:717-725(1986) [PubMed] [Europe PMC] [Abstract]
"A novel arginine (245) to glutamine change in exon 8 of the ornithine carbamoyl transferase gene in two unrelated children presenting with late onset deficiency and showing the same enzymatic pattern."
Hum. Mol. Genet. 3:831-832(1994) [PubMed] [Europe PMC] [Abstract]
"Lysine 88 acetylation negatively regulates ornithine carbamoyltransferase activity in response to nutrient signals."
J. Biol. Chem. 284:13669-13675(2009) [PubMed] [Europe PMC] [Abstract]
"An enzyme assisted RP-RPLC approach for in-depth analysis of human liver phosphoproteome."
J. Proteomics 96:253-262(2014) [PubMed] [Europe PMC] [Abstract]
"1.85-A resolution crystal structure of human ornithine transcarbamoylase complexed with N-phosphonacetyl-L-ornithine. Catalytic mechanism and correlation with inherited deficiency."
J. Biol. Chem. 273:34247-34254(1998) [PubMed] [Europe PMC] [Abstract]
"Crystal structure of human ornithine transcarbamylase complexed with carbamoyl phosphate and L-norvaline at 1.9 A resolution."
Proteins 39:271-277(2000) [PubMed] [Europe PMC] [Abstract]
"Mutations and polymorphisms in the human ornithine transcarbamylase gene."
Hum. Mutat. 2:174-178(1993) [PubMed] [Europe PMC] [Abstract]
"Mutations and polymorphisms in the human ornithine transcarbamylase gene: mutation update addendum."
Hum. Mutat. 5:293-295(1995) [PubMed] [Europe PMC] [Abstract]
"The molecular basis of ornithine transcarbamylase deficiency: modelling the human enzyme and the effects of mutations."
J. Med. Genet. 32:680-688(1995) [PubMed] [Europe PMC] [Abstract]
"Characterization of point mutations in the same arginine codon in three unrelated patients with ornithine transcarbamylase deficiency."
J. Clin. Invest. 82:1353-1358(1988) [PubMed] [Europe PMC] [Abstract]
"Scanning detection of mutations in human ornithine transcarbamoylase by chemical mismatch cleavage."
Proc. Natl. Acad. Sci. U.S.A. 86:5888-5892(1989) [PubMed] [Europe PMC] [Abstract]
"Use of denaturing gradient gel electrophoresis for detection of mutation and prospective diagnosis in late onset ornithine transcarbamylase deficiency."
Genomics 7:167-172(1990) [PubMed] [Europe PMC] [Abstract]
"Improved molecular diagnostics for ornithine transcarbamylase deficiency."
Am. J. Hum. Genet. 48:212-222(1991) [PubMed] [Europe PMC] [Abstract]
"Fatal hyperammonemia resulting from a C-to-T mutation at a MspI site of the ornithine transcarbamylase gene."
Hum. Genet. 88:153-156(1991) [PubMed] [Europe PMC] [Abstract]
"Six new mutations in the ornithine transcarbamylase gene detected by single-strand conformational polymorphism."
Pediatr. Res. 32:600-604(1992) [PubMed] [Europe PMC] [Abstract]
"Single-strand conformational polymorphism and direct sequencing applied to carrier testing in families with ornithine transcarbamylase deficiency."
Hum. Genet. 91:321-325(1993) [PubMed] [Europe PMC] [Abstract]
"The ornithine transcarbamylase gene: new 'private' mutations in four patients and study of a polymorphism."
Hum. Mutat. 3:318-320(1994) [PubMed] [Europe PMC] [Abstract]
"Four newly identified ornithine transcarbamylase (OTC) mutations (D126G, R129H, I172M and W332X) in Japanese male patients with early-onset OTC deficiency."
Hum. Mutat. 3:402-406(1994) [PubMed] [Europe PMC] [Abstract]
"Seven new mutations in the human ornithine transcarbamylase gene."
Hum. Mutat. 4:57-60(1994) [PubMed] [Europe PMC] [Abstract]
"A splicing mutation, a nonsense mutation (Y167X) and two missense mutations (I159T and A209V) in Spanish patients with ornithine transcarbamylase deficiency."
Hum. Genet. 96:549-551(1995) [PubMed] [Europe PMC] [Abstract]
"A novel point mutation at codon 269 of the ornithine transcarbamylase (OTC) gene causing neonatal onset of OTC deficiency."
J. Inherit. Metab. Dis. 18:356-357(1995) [PubMed] [Europe PMC] [Abstract]
"Partial duplication [dup. TCAC (178)] and novel point mutations (T125M, G188R, A209V, and H302L) of the ornithine transcarbamylase gene in congenital hyperammonemia."
Hum. Mutat. 8:74-76(1996) [PubMed] [Europe PMC] [Abstract]
"Genotype-phenotype correlations in ornithine transcarbamylase deficiency."
Enzyme Protein 49:191-191(1996)
"Ornithine transcarbamylase deficiency: characterization of gene mutations and polymorphisms."
Hum. Mutat. 8:333-339(1996) [PubMed] [Europe PMC] [Abstract]
"A 3-base pair in-frame deletion in exon 8 (delGlu272/273) of the ornithine transcarbamylase gene in late-onset hyperammonemic coma."
Hum. Mutat. 8:373-374(1996) [PubMed] [Europe PMC] [Abstract]
"Identification of new mutations in the ornithine transcarbamylase (OTC) gene in Korean families."
J. Inherit. Metab. Dis. 19:31-42(1996) [PubMed] [Europe PMC] [Abstract]
"The ornithine transcarbamylase (OTC) gene: mutations in 50 Japanese families with OTC deficiency."
Am. J. Med. Genet. 71:378-383(1997) [PubMed] [Europe PMC] [Abstract]
"Expression, purification and kinetic characterization of wild-type human ornithine transcarbamylase and a recurrent mutant that produces 'late onset' hyperammonaemia."
Biochem. J. 322:625-631(1997) [PubMed] [Europe PMC] [Abstract]
"Ornithine transcarbamylase deficiency: ten new mutations and high proportion of de novo mutations in heterozygous females."
Hum. Mutat. 9:409-411(1997) [PubMed] [Europe PMC] [Abstract]
"Identification of 'private' mutations in patients with ornithine transcarbamylase deficiency."
J. Inherit. Metab. Dis. 20:525-527(1997) [PubMed] [Europe PMC] [Abstract]
"Ten novel mutations of the ornithine transcarbamylase (OTC) gene in OTC deficiency."
Hum. Mutat. Suppl. 1:S5-S7(1998) [PubMed] [Europe PMC] [Abstract]
"Novel intragenic deletions and point mutations of the ornithine transcarbamylase gene in congenital hyperammonemia."
Hum. Mutat. Suppl. 1:S81-S84(1998) [PubMed] [Europe PMC] [Abstract]
"Y55D mutation in ornithine transcarbamylase associated with late-onset hyperammonemia in a male."
Hum. Mutat. Suppl. 1:S131-S133(1998) [PubMed] [Europe PMC] [Abstract]
"A novel missense mutation in the human ornithine transcarbamylase gene."
Hum. Mutat. 12:220-220(1998)
"Identification of a cytogenetic deletion and of four novel mutations (Q69X, I172F, G188V, G197R) affecting the gene for ornithine transcarbamylase (OTC) in Spanish patients with OTC deficiency."
Hum. Mutat. 14:352-353(1999) [PubMed] [Europe PMC] [Abstract]
"Three novel and one recurrent ornithine carbamoyltransferase gene mutations in Polish patients."
J. Inherit. Metab. Dis. 22:92-93(1999) [PubMed] [Europe PMC] [Abstract]
"Lymphocyte mRNA analysis of the ornithine transcarbamylase gene in Italian OTCD male patients and manifesting carriers: identification of novel mutations."
Hum. Mutat. 15:380-381(2000) [PubMed] [Europe PMC] [Abstract]
"Identification of seven novel missense mutations, two splice-site mutations, two microdeletions and a polymorphic amino acid substitution in the gene for ornithine transcarbamylase (OTC) in patients with OTC deficiency."
Hum. Mutat. 19:185-186(2002) [PubMed] [Europe PMC] [Abstract]
"The consensus coding sequences of human breast and colorectal cancers."
Science 314:268-274(2006) [PubMed] [Europe PMC] [Abstract]