OTC antibody (AA 51-150)

Catalog No. ABIN720505

Product Details anti-OTC Antibody

Target: OTC (Ornithine Carbamoyltransferase (OTC))

Binding Specificity: AA 51-150

Reactivity: Human

Host: Rabbit

Clonality: Polyclonal

Conjugate: This OTC antibody is un-conjugated

Application: Western Blotting (WB), ELISA

Cross-Reactivity: Human

Predicted Reactivity: Mouse,Rat,Dog,Cow,Pig,Horse

Purification: Purified by Protein A.

Immunogen: KLH conjugated synthetic peptide derived from human OTC

Isotype: IgG

Application Details

Application Notes: WB 1:300-5000
ELISA 1:500-1000

Restrictions: For Research Use only

Handling

Format: Liquid

Concentration: 1 μg/μL

Buffer: 0.01M TBS( pH 7.4) with 1 % BSA, 0.02 % Proclin300 and 50 % Glycerol.

Preservative: ProClin

Precaution of Use: This product contains ProClin: a POISONOUS AND HAZARDOUS SUBSTANCE, which should be handled by trained staff only.

Storage: 4 °C,-20 °C

Storage Comment: Shipped at 4°C. Store at -20°C for one year. Avoid repeated freeze/thaw cycles.

Expiry Date: 12 months

Target Details for OTC

Target: OTC (Ornithine Carbamoyltransferase (OTC))

Alternative Name: OTC (OTC Products)

Background:

Synonyms: OCTD, Ornithine carbamoyltransferase, mitochondrial, Ornithine transcarbamylase, OTCase, OTC

Background: 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.

Gene ID: 5009

UniProt: P00480