Ornithine carbamoyltransferase Recombinant Protein | OTC recombinant protein

Recombinant mouse Ornithine carbamoyltransferase, mitochondrial

Cat. Num. AAA9420517

• Gene Names: Otc; Sf; spf; AI265390
• Reactivity: Mouse
• Purity: Greater than 90% as determined by SDS-PAGE.
• Synonyms: Ornithine carbamoyltransferase; Recombinant mouse Ornithine carbamoyltransferase; mitochondrial; Ornithine transcarbamylase; OTCase; OTC recombinant protein

• Host: E Coli
• Reactivity: Mouse
• Purity/Purification: Greater than 90% as determined by SDS-PAGE.
• Form/Format: 20mM Tris-HCl based buffer, pH8.0
• Sequence Positions: Full Length, 33-354aa
• Sequence: SQVQLKGRDLLTLKNFTGEEIQYMLWLSADLKFRIKQKGEYLPLLQGKSLGMIFEKRSTRTRLSTETGFALLGGHPSFLTTQDIHLGVNESLTDTARVLSSMTDAVLARVYKQSDLDTLAKEASIPIVNGLSDLYHPIQILADYLTLQEHYGSLKGLTLSWIGDGNNILHSIMMSAAKFGMHLQAATPKGYEPDPNIVKLAEQYAKENGTKLSMTNDPLEAARGGNVLITDTWISMGQEDEKKKRLQAFQGYQVTMKTAKVAASDWTFLHCLPRKPEEVDDEVFYSPRSLVFPEAENRKWTIMAVMVSLLTDYSPVLQKPKF

• Tag Info: His-SUMO-tag
• 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.

NCBI and Uniprot Product Information

• NCBI GI #: 6679184
• NCBI GeneID: 18416
• NCBI Accession #: NP_032795.1
• NCBI GenBank Nucleotide #: NM_008769.4
• UniProt Accession #: P11725
• Molecular Weight: 52.1kD
• NCBI Official Full Name: ornithine carbamoyltransferase, mitochondrial
• NCBI Official Synonym Full Names: ornithine transcarbamylase
• NCBI Official Symbol: Otc
• NCBI Official Synonym Symbols: Sf; spf; AI265390
• NCBI Protein Information: ornithine carbamoyltransferase, mitochondrial
• UniProt Protein Name: Ornithine carbamoyltransferase, mitochondrial
• Protein Family: Ornithine carbamoyltransferase
• UniProt Gene Name: Otc
• UniProt Synonym Gene Names: OTCase

Uniprot Description

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