OTC Antikörper (AA 51-150)

Produktnummer ABIN720505

Produktdetails anti-OTC Antikörper

Target: OTC (Ornithine Carbamoyltransferase (OTC))

Bindungsspezifität: AA 51-150

Reaktivität: Human

Wirt: Kaninchen

Klonalität: Polyklonal

Konjugat: Dieser OTC Antikörper ist unkonjugiert

Applikation: Western Blotting (WB), ELISA

Kreuzreaktivität: Human

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

Aufreinigung: Purified by Protein A.

Immunogen: KLH conjugated synthetic peptide derived from human OTC

Isotyp: IgG

Anwendungsinformationen

Applikationshinweise: WB 1:300-5000
ELISA 1:500-1000

Beschränkungen: Nur für Forschungszwecke einsetzbar

Handhabung

Format: Liquid

Konzentration: 1 μg/μL

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

Konservierungsmittel: ProClin

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

Lagerung: 4 °C,-20 °C

Informationen zur Lagerung: Shipped at 4°C. Store at -20°C for one year. Avoid repeated freeze/thaw cycles.

Haltbarkeit: 12 months

Details zu OTC

Target: OTC (Ornithine Carbamoyltransferase (OTC))

Andere Bezeichnung: OTC (OTC Produkte)

Hintergrund:

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.

Gen-ID: 5009

UniProt: P00480