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

Ornithine Carbamoyltransferase (OTC, Ornithine Transcarbamylase, OTCase, MGC129967, MGC129968, MGC138856, OCTD) (Biotin)

Cat. Num. AAA6491125

• Gene Names: OTC; OCTD
• Reactivity: Human
• Applications: Western Blot, ELISA
• Purity: Purified by Protein A Affinity Chromatography.
• Synonyms: Ornithine Carbamoyltransferase; Polyclonal Antibody; Ornithine Carbamoyltransferase (OTC; Ornithine Transcarbamylase; OTCase; MGC129967; MGC129968; MGC138856; OCTD) (Biotin); mitochondrial; EC=2.1.3.3; anti-OTC antibody

• Host: Rabbit
• Reactivity: Human
• Clonality: Polyclonal
• Isotype: IgG
• Specificity: Recognizes human OTC at ~40kD.
• Purity/Purification: Purified by Protein A Affinity Chromatography.
• Form/Format: Supplied as a liquid in PBS, pH 7.2. No preservative added. Labeled with Biotin.

• Applicable Applications for anti-OTC antibody: Western Blot (WB), ELISA (EIA)
• Application Notes: WB: 1:100-1:500; ELISA: 1:1,000; Applications are based on unconjugated antibody.
• Immunogen: KLH-conjugated synthetic peptide mapping to a fragment of residues within amino acids 71-98 in the central region of human OTC, UniProt Accession #NP_000522.3, P00480. Species sequence homology: mouse, rat, bovine, porcine
• Conjugate: Biotin
• Note: Preservative Free
• Preparation and Storage: Store product at 4 degree C if to be used immediately within two weeks. For long-term storage, aliquot to avoid repeated freezing and thawing and store at -20 degree C. Aliquots are stable at -20 degree C for 12 months after receipt. Dilute required amount only prior to immediate use. Further dilutions can be made in assay buffer. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.

Western Blot (WB)

(Western Blot analysis in HepG2 cell line lysates (35ug/lane) usingMBS629248. OTC (arrow) was detected using the purified Pab.)

Immunohistochemistry (IHC)

(Immunohistochemistry analysis in formalin-fixed and paraffin-embedded human hepatoarcinoma usingMBS629248, which was peroxidase-conjugated to the secondary antibody, followed by DAB staining. This data demonstrates the use of MBS629248 for immunohistochemistry.)

Flow Cytometry (FC/FACS)

(Flow Cytometric analysis of ATDC5 cells using MBS629248 (bottom histogram) compared to a negative control cell (top histogram). FITC-conjugated goat-anti-rabbit secondary antibodies were used for the analysis.)

Product Categories/Family for anti-OTC antibody

Antibodies; Enzymes

References

1. Hansmannel, F., et.al., Neurosci. Lett. 449(1), 76-80 (2009).

NCBI and Uniprot Product Information

• NCBI GI #: 38788445
• NCBI GeneID: 5009
• NCBI Accession #: NP_000522.3
• NCBI GenBank Nucleotide #: NM_000531.5
• UniProt Accession #: P00480; Q3KNR1; Q6B0I1; Q9NYJ5; A8K9P2; D3DWB0
• Molecular Weight: 39,935 Da
• NCBI Official Full Name: ornithine carbamoyltransferase, mitochondrial
• NCBI Official Synonym Full Names: ornithine carbamoyltransferase
• NCBI Official Symbol: OTC
• NCBI Official Synonym Symbols: OCTD
• NCBI Protein Information: ornithine carbamoyltransferase, mitochondrial; OTCase; ornithine transcarbamylase
• UniProt Protein Name: Ornithine carbamoyltransferase, mitochondrial
• Protein Family: Ornithine carbamoyltransferase
• UniProt Gene Name: OTC
• UniProt Synonym Gene Names: OTCase
• UniProt Entry Name: OTC_HUMAN

NCBI Description

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 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; 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