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Product name
Ornithine Carbamoyltransferase protein (Human)
See all Ornithine Carbamoyltransferase products (2) ...
Protein description
Recombinant full length Human Ornithine Carbamoyltransferase (amino acids 1-354) 40 kDa. (UniProt ID = P00480).
Uniprot accession
P00480
Molecular weight
40kDa
Protein length
354 amino acids
Expression host
E. coli
Properties
Purity
by SDS-PAGE
E.C. Number
2.1.3.3
Form
Liquid
Storage instructions
Shipped on dry ice. Upon delivery aliquot and store at -80ºC. Avoid freeze / thaw cycles.
Storage buffer
Constituents: 0.79% Tris HCl, 0.15% EDTA, 10% Glycerol, 0.88% Sodium chloride
Concentration
Concentration information loading...
Sequence notes
MLFNLRILLN NAAFRNGHNF MVRNFRCGQP LQNKVQLKGR DLLTLKNFTG EEIKYMLWLS ADLKFRIKQK GEYLPLLQGK SLGMIFEKRS TRTRLSTETG FALLGGHPCF LTTQDIHLGV NESLTDTARV LSSMADAVLA RVYKQSDLDT LAKEASIPII NGLSDLYHPI QILADYLTLQ EHYSSLKGLT LSWIGDGNNI LHSIMMSAAK FGMHLQAATP KGYEPDASVT KLAEQYAKEN GTKLLLTNDP LEAAHGGNVL ITDTWISMGQ EEEKKKRLQA FQGYQVTMKT AKVAASDWTF LHCLPRKPEE VDDEVFYSPR SLVFPEAENR KWTIMAVMVS LLTDYSPQLQKPKF
Research areas
Metabolism >> Metabolic signalling pathways >> Amino acid metabolism
Metabolism >> Mitochondrial Metabolism >> Mitochondrial markers
Signal Transduction >> Metabolism >> Mitochondrial
Signal Transduction >> Metabolism >> Amino Acids
Tags & Cell Markers >> Subcellular Markers >> Organelles >> Mitochondria
Applications
Show applications key
Our Abpromise guarantee covers the use of ab123452 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
SDS-PAGE: Use at an assay dependent concentration.
WB: Use at an assay dependent concentration. Predicted molecular weight: 40 kDa.
ELISA: Use at an assay dependent concentration.
Protein info
Tissue specificity
Mainly expressed in liver and intestinal mucosa.
Pathway
Nitrogen metabolism; urea cycle; L-citrulline from L-ornithine and carbamoyl phosphate: step 1/1.
Involvement in disease
Defects in OTC are the cause of ornithine carbamoyltransferase deficiency (OTCD) [MIM:311250]. 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.
Sequence similarities
Belongs to the ATCase/OTCase family.
Cellular localization
Mitochondrion matrix.
Target information above from: UniProt accessionP00480
The UniProt Consortium
The Universal Protein Resource (UniProt) in 2010
Nucleic Acids Res. 38:D142-D148 (2010).
Ornithine Carbamoyltransferase protein (Human) images:
SDS-PAGE - Ornithine Carbamoyltransferase protein (Human) (ab123452)
10-20% acrylamide separation gel showing ab123452 at approximately 40kDa (1µg).
Western blot - Ornithine Carbamoyltransferase protein (Human) (ab123452)
Anti-Ornithine Carbamoyltransferase antibody (ab86665) at 0.5 µg/ml +
Secondary
Goat polyclonal to Rabbit IgG – H&L – Pre-Adsorbed (HRP) at 1/10000 dilution
developed using the ECL technique
Predicted band size : 40 kDa
Observed band size : 40 kDa
Exposure time : 5 minutes
References for Ornithine Carbamoyltransferase protein (Human) (ab123452)
ab123452 has not yet been referenced specifically in any publications.
Publishing research using ab123452? Please let us know so that we can cite the reference in this datasheet
Please note: All products are "FOR RESEARCH USE ONLY AND ARE NOT INTENDED FOR DIAGNOSTIC OR THERAPEUTIC USE"