Rabbit anti-Human OTC/Ornithine Carbamoyltransferase Polyclonal Antibody | anti-OTC antibody

Anti-OTC/Ornithine Carbamoyltransferase Antibody, Rabbit Polyclonal

Cat. Num. AAA258815

• Reactivity: Human
• Applications: Immunohistochemistry
• Purity: Protein A & Antigen Affinity
• Synonyms: OTC/Ornithine Carbamoyltransferase, Antibody; Anti-OTC/Ornithine Carbamoyltransferase Antibody, Rabbit Polyclonal; ornithine carbamoyltransferase; Anti-OCTD Antibody; anti-OTC antibody

• Host: Rabbit
• Reactivity: Human
• Clonality: Polyclonal
• Isotype: Rabbit IgG
• Specificity: Human OTC/Ornithine Carbamoyltransferase
• Purity/Purification: Protein A & Antigen Affinity
• Form/Format: Liquid; 0.2um filtered solution in PBS

• Applicable Applications for anti-OTC antibody: IHC (Immunohistochemistry)
• Immunogen: A synthetic peptide corresponding to the center region of the Human OTC/Ornithine Carbamoyltransferase
• Preparation: Produced in rabbits immunized with a synthetic peptide corresponding to the center region of the Human OTC/Ornithine Carbamoyltransferase, and purified by antigen affinity chromatography.
• Preparation and Storage: This antibody can be stored at 2-8 degree C for one month without detectable loss of activity. Antibody products are stable for twelve months from date of receipt when stored at -20 degree C to -80 degree C. Avoid repeated freeze-thaw cycles.

IHC (Immunohiostchemistry)

(Immunochemical staining of human OTC in human small intestine with rabbit polyclonal antibody at 1:200 dilution, formalin-fixed paraffin embedded sections.)

IHC (Immunohistochemistry)

(Immunochemical staining of human OTC in human liver with rabbit polyclonal antibody at 1:200 dilution, formalin-fixed paraffin embedded sections.)

Related Product Information for anti-OTC antibody

OTC (Ornithine Carbamoyltransferase) is a Protein Coding gene. The OTC gene, located on Xp11.4, is conserved in dog, cow, mouse, and rat, etc. This nuclear gene encodes a mitochondrial matrix enzyme. Missense, nonsense, and frameshift mutations in this enzyme lead to ornithine transcarbamylase deficiency, which causes hyperammonemia. OTC deficiency is the most common urea cycle disorder and is inherited in an X-linked semi-dominant trait, and the OTC gene maps to Xp21. Since the gene for this enzyme maps close to that for Duchenne muscular dystrophy, it may play a role in that disease also. Diseases associated with OTC include Ornithine Transcarbamylase Deficiency, Hyperammonemia Due To and Disorder Of Ornithine Metabolism. Among its related pathways are Arginine biosynthesis and Viral mRNA Translation.

References

Wang L, et al. (2017) Aav gene therapy corrects otc deficiency and prevents liver fibrosis in aged otc-knock out heterozygous mice. Mol Genet Metab 120 (4): 299-305.Shchelochkov OA, et al. (2009) High-frequency detection of deletions and variable rearrangements at the ornithine transcarbamylase (otc) locus by oligonucleotide array cgh. Mol Genet Metab 96 (3): 97-105.Lichter-Konecki U, et al. (1993) Ornithine transcarbamylase deficiency. In: Adam MP, Ardinger HH, Pagon RA, Wallace SE, Bean LJH, Stephens K, Amemiya A (eds) Genereviews(). University of Washington, SeattleJoost K, et al. (2011) Whole xp deletion in a girl with mental retardation, epilepsy, and biochemical features of otc deficiency. Mol Syndromol 1 (6): 311-315.