Product Name
Ornithine carbamoyltransferase (OTC), Recombinant Protein
Popular Item
Full Product Name
Recombinant mouse Ornithine carbamoyltransferase, mitochondrial
Product Synonym Names
Ornithine transcarbamylase; OTCase
Product Gene Name
OTC recombinant protein
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Research Use Only
For Research Use Only. Not for use in diagnostic procedures.
Sequence Positions
Full Length, 33-354aa
Sequence
SQVQLKGRDL LTLKNFTGEE IQYMLWLSAD LKFRIKQKGE YLPLLQGKSL GMIFEKRSTR TRLSTETGFA LLGGHPSFLT TQDIHLGVNE SLTDTARVLS SMTDAVLARV YKQSDLDTLA KEASIPIVNG LSDLYHPIQI LADYLTLQEH YGSLKGLTLS WIGDGNNILH SIMMSAAKFG MHLQAATPKG YEPDPNIVKL AEQYAKENGT KLSMTNDPLE AARGGNVLIT DTWISMGQED EKKKRLQAFQ GYQVTMKTAK VAASDWTFLH CLPRKPEEVD DEVFYSPRSL VFPEAENRKW TIMAVMVSLL TDYSPVLQKP KF
3D Structure
ModBase 3D Structure for P11725
Purity/Purification
Greater than 90% as determined by SDS-PAGE.
Form/Format
20mM Tris-HCl based buffer, pH8.0
Preparation and Storage
Store at -20°C, for extended storage, conserve at -20°C or -80°C. Repeated freezing and thawing is not recommended. Store working aliquots at 4°C for up to one week.
Other Notes
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.
NCBI/Uniprot data below describe general gene information for OTC. It may not necessarily be applicable to this product.
NCBI Accession #
NP_032795.1
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NCBI GenBank Nucleotide #
NM_008769.4
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UniProt Primary Accession #
P11725
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UniProt Related Accession #
P11725[Other Products]
NCBI Official Full Name
ornithine carbamoyltransferase, mitochondrial
NCBI Official Synonym Full Names
ornithine transcarbamylase
NCBI Official Symbol
Otc??[Similar Products]
NCBI Official Synonym Symbols
Sf; spf; AI265390
??[Similar Products]
NCBI Protein Information
ornithine carbamoyltransferase, mitochondrial
UniProt Protein Name
Ornithine carbamoyltransferase, mitochondrial
UniProt Synonym Protein Names
Ornithine transcarbamylase; OTCase
Protein Family
Ornithine carbamoyltransferase
UniProt Gene Name
Otc??[Similar Products]
UniProt Synonym Gene Names
OTCase??[Similar Products]
UniProt Comments for OTC
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; EC 2.1.3.3; Mitochondrial; Transferase
Chromosomal Location of Human Ortholog: X A1.1|X 4.66 cM
Cellular Component: cytoplasm; mitochondrial inner membrane; mitochondrion
Molecular Function: amino acid binding; ornithine carbamoyltransferase activity; phosphate binding; phospholipid binding
Biological Process: anion homeostasis; arginine biosynthetic process via ornithine; citrulline biosynthetic process; ornithine catabolic process; ornithine metabolic process; urea cycle
Research Articles on OTC
1. Gene correction in ***** OTC-deficient mice was lower and accompanied by larger deletions that ablated residual expression from the endogenous OTC gene, leading to diminished protein tolerance and lethal hyperammonemia on a chow diet
Precautions
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Disclaimer
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