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Anti-Ornithine Carbamoyltransferase/OTC Antibody Picoband® Fluoro647 Conjugated

Selected Variant

A00721-1-Fluoro647

Size: 100 µg/Vial

Product Specifications

Background

Ornithine transcarbamylase (OTC) (also called ornithine carbamoyltransferase) is an enzyme that catalyzes the reaction between carbamoyl phosphate (CP) and ornithine (Orn) to form citrulline (Cit) and phosphate (Pi) . 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 also play a role in that disease.

Synonyms

Ornithine carbamoyltransferase, mitochondrial;2.1.3.3; Ornithine transcarbamylase; OTCase; OTC

Gene Name

OTC

Gene ID

5009

UniProt

P00480

Host

Rabbit

Reactivity

Human, Mouse, Rat

Cross Reactivity

No cross-reactivity with other proteins.

Immunogen

A synthetic peptide corresponding to a sequence at the N-terminus of human OTC, different from the related mouse and rat sequences by three amino acids.

Clonality

Polyclonal

Tissue Specificity

Mainly expressed in liver and intestinal mucosa.

Applications

Flow Cytometry

Field of Research

Biochemicals, Chemical Type, Neuroscience, Neurotransmission, Obesity, Receptors / Channels

Purification

Immunogen affinity purified.

Form

Liquid

Function

May be a tumor suppressor. May be involved in energy and/or nutrient sensing through the AMPK and mTOR signaling pathways. May regulate phosphorylation of RPS6KB1.

References & Citations

1. Langley DB, Templeton MD, Fields BA, Mitchell RE, Collyer CA (June 2000) . Mechanism of inactivation of ornithine transcarbamoylase by Ndelta - (N'-Sulfodiaminophosphinyl) -L-ornithine, a true transition state analogue? Crystal structure and implications for catalytic mechanism. J. Biol. Chem. 275 (26) : 20012–9. 2. Nagamine T, Saito S, Kaneko M, Sekiguchi T, Sugimoto H, Takehara K, Takagi H (June 1995) . Effect of biotin on ammonia intoxication in rats and mice. J. Gastroenterol. 30 (3) : 351–5.

Storage Conditions

At -20 ̊C for one year from date of receipt. Avoid repeated freezing and thawing. Protect from light.

Calculated Molecular Weight

39935 MW

Specificity

No cross reactivity with other proteins.

Applications Notes

6

Gene Name Synonym

Ornithine carbamoyltransferase, mitochondrial

Subcellular Location

Mitochondrion matrix.

Protein Name

Ornithine carbamoyltransferase, mitochondrial

Isotype

Rabbit IgG

Contents

Each vial contains 50% glycerol, 0.9% NaCl, 0.2% Na2HPO4, 0.02% NaN3.

Price

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