OTC anticorps (AA 51-150)

N° du produit ABIN720505

Détail du produit anti-OTC anticorps

Antigène: OTC (Ornithine Carbamoyltransferase (OTC))

Épitope: AA 51-150

Reactivité: Humain

Hôte: Lapin

Clonalité: Polyclonal

Conjugué: Cet anticorp OTC est non-conjugé

Application: Western Blotting (WB), ELISA

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 (OTC Produits)

Synonymes: anticorps OCTD, anticorps 2810428A13Rik, anticorps AA589422, anticorps AW457381, anticorps OCT, anticorps Plxn2, anticorps mKIAA0463, anticorps F1B16.13, anticorps F1B16_13, anticorps ORNITHINE CARBAMOYLTRANSFERASE, anticorps ornithine carbamoyltransferase, anticorps BA4351, anticorps PSPTO4164, anticorps PLXN2, anticorps AI265390, anticorps Sf, anticorps spf, anticorps si:dkey-19h21.3, anticorps ornithine carbamoyltransferase, anticorps plexin A2, anticorps ornithine carbamoyltransferase ArgF, anticorps ornithine transcarbamylase, anticorps OTC, anticorps Plxna2, anticorps Otc, anticorps argF, anticorps argF-2, anticorps atpD-2, anticorps CNC04300, anticorps PLXNA2, anticorps 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