Recombinant Human Ornithine Carbamoyltransferase | MyBioSource MBS144835 product information

product summary

company name :

MyBioSource

product type :

protein

product name :

Recombinant Human Ornithine Carbamoyltransferase

catalog :

MBS144835

quantity :

0.002 mg

price :

140 USD

more info or order :

MyBioSource product webpage

product information

catalog number :

MBS144835

products type :

Recombinant Protein

products full name :

Recombinant Human Ornithine Carbamoyltransferase

products short name :

Ornithine Carbamoyltransferase

products name syn :

Ornithine carbamoyltransferase mitochondrial, Ornithine transcarbamylase, OTCase, OCTD, EC 2.1.3.3.

other names :

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

products gene name :

OTC

other gene names :

OTC; OTC; OCTD; OTCase

uniprot entry name :

OTC_HUMAN

host :

E Coli

sequence length :

354

sequence :

MGSSHHHHHH SSGLVPRGSH MGSHMNKVQL KGRDLLTLKN FTGEEIKYML WLSADLKFRI KQKGEYLPLL QGKSLGMIFE KRSTRTRLST ETGFALLGGH PCFLTTQDIH LGVNESLTDT ARVLSSMADA VLARVYKQSD LDTLAKEASI PIINGLSDLY HPIQILADYL TLQEHYSSLK GLTLSWIGDG NNILHSIMMS AAKFGMHLQA ATPKGYEPDA SVTKLAEQYA KENGTKLLLT NDPLEAAHGG NVLITDTWIS MGQEEEKKKR LQAFQGYQVT MKTAKVAASD WTFLHCLPRK PEEVDDEVFY SPRSLVFPEA ENRKWTIMAV MVSLLTDYSP QLQKPKF.

purity :

Greater than 90% as determined by SDS-PAGE.

form :

The OTC solution (0.5mg/ml) contains 20mM MES buffer (pH 6.0), 100mM Nacl, 2mM DTT and 10% glycerol.

storage stability :

Store at 4 degree C if entire vial will be used within 2-4 weeks. Store, frozen at -20 degree C for longer periods of time. For long term storage it is recommended to add a carrier protein (0.1% HSA or BSA). Avoid multiple freeze-thaw cycles.

products categories :

ENZYMES; Enzymes; Transferase

products description :

Description: OTC Recombinant produced in E Coli is a single polypeptide chain containing 347 amino acids (33-354) and having a molecular mass of 38.9kDa.OTC is fused to a 25 amino acid His-tag at N-terminus & purified by proprietary chromatographic techniques. Introduction: OTC is a member of the ATCase/OTCase family. OTC has a key part in the urea cycle, catalyzing the second step in this pathway: the transformation of L-orthinine and carbamoyl phosphate to L-citrulline. In humans, the urea cycle is a vital pathway to detoxification of ammonia. Alterations in the gene encoding OTC are linked to the X-linked disorder OTCD (ornithine carbamoyltransferase deficiency). OTCD disorder of the urea cycle is characterized by hyperammonemia.

ncbi gi num :

38788445

ncbi acc num :

NP_000522.3

ncbi gb acc num :

NM_000531.5

uniprot acc num :

P00480

ncbi mol weight :

39,935 Da

ncbi pathways :

Arginine And Proline Metabolism Pathway (82957); Arginine And Proline Metabolism Pathway (323); Biosynthesis Of Amino Acids Pathway (790012); Biosynthesis Of Amino Acids Pathway (795174); Metabolic Pathways (132956); Metabolism Pathway (477135); Metabolism Of Amino Acids And Derivatives Pathway (106169); Urea Cycle Pathway (413351); Urea Cycle Pathway (106171); Urea Cycle Pathway (468222)

ncbi summary :

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]

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: Amino Acid Metabolism - arginine and proline; Mitochondrial; Transferase; EC 2.1.3.3. 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; response to insulin stimulus; urea cycle. Disease: Ornithine Transcarbamylase Deficiency, Hyperammonemia Due To

size1 :

0.002 mg

price1 :

140 USD

size2 :

0.01 mg

price2 :

205

size3 :

1 mg

price3 :

5015

more info or order :

MyBioSource product webpage