OTC anticorps (AA 51-150)

N° du produit ABIN720505

Cet anticorps Lapin Polyclonal détecte spécifiquement OTC dans WB et ELISA. Il présente une réactivité envers Humain.

Aperçu rapide pour OTC anticorps (AA 51-150) (ABIN720505)

Antigène: OTC (Ornithine Carbamoyltransferase (OTC))

Reactivité: Humain

Hôte: Lapin

Clonalité: Polyclonal

Conjugué: Cet anticorp OTC est non-conjugé

Application: Western Blotting (WB), ELISA

Détail du produit anti-OTC anticorps

Épitope: AA 51-150

Réactivité croisée: Humain

Homologie: Mouse,Rat,Dog,Cow,Pig,Horse

Purification: Purified by Protein A.

Immunogène: KLH conjugated synthetic peptide derived from human OTC

Isotype: IgG

Information d'application

Indications d'application: WB 1:300-5000
ELISA 1:500-1000

Restrictions: For Research Use only

Stockage

Format: Liquid

Concentration: 1 μg/μL

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

Agent conservateur: ProClin

Précaution d'utilisation: This product contains ProClin: a POISONOUS AND HAZARDOUS SUBSTANCE, which should be handled by trained staff only.

Stock: 4 °C,-20 °C

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

Date de péremption: 12 months

Détails sur OTC

Antigène: OTC (Ornithine Carbamoyltransferase (OTC))

Autre désignation: OTC

Sujet:

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.

ID gène: 5009

UniProt: P00480