ETHE1 Antikörper (AA 191-254) (AbBy Fluor® 594)

Produktnummer ABIN2812956

Dieses Kaninchen Polyklonal-Antikörper erkennt spezifisch ETHE1 in IF (cc) und IF (p). Er zeigt eine Reaktivität gegenüber Human.

Kurzübersicht für ETHE1 Antikörper (AA 191-254) (AbBy Fluor® 594) (ABIN2812956)

Target: ETHE1 (Ethylmalonic Encephalopathy 1 (ETHE1))

Reaktivität: Human

Wirt: Kaninchen

Klonalität: Polyklonal

Konjugat: Dieser ETHE1 Antikörper ist konjugiert mit AbBy Fluor® 594

Applikation: Immunofluorescence (Cultured Cells) (IF (cc)), Immunofluorescence (Paraffin-embedded Sections) (IF (p))

Produktdetails anti-ETHE1 Antikörper (AbBy Fluor® 594)

Bindungsspezifität: AA 191-254

Homologie: Human,Mouse,Dog,Cow,Sheep,Pig,Rabbit

Aufreinigung: Purified by Protein A.

Immunogen: KLH conjugated synthetic peptide derived from human E1

Isotyp: IgG

Anwendungsinformationen

Applikationshinweise: IF(IHC-P) 1:50-200
IF(IHC-F) 1:50-200
IF(ICC) 1:50-200

Beschränkungen: Nur für Forschungszwecke einsetzbar

Handhabung

Format: Liquid

Konzentration: 1 μg/μL

Buffer: Aqueous buffered solution containing 0.01M TBS ( pH 7.4) with 1 % BSA, 0.03 % 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: -20 °C

Informationen zur Lagerung: Store at -20°C. Aliquot into multiple vials to avoid repeated freeze-thaw cycles.

Haltbarkeit: 12 months

Details zu ETHE1

Target: ETHE1 (Ethylmalonic Encephalopathy 1 (ETHE1))

Andere Bezeichnung: ETHE1

Hintergrund:

Synonyms: mitochondrial, Ethe1, ETHE1 protein, mitochondrial precursor, ETHE1_HUMAN, ethylmalonic encephalopathy 1, Ethylmalonic encephalopathy protein 1, hepatoma subtracted clone one, Hepatoma subtracted clone one protein, HSCO, Protein ETHE1, YF13H12.

Background: Probably plays an important role in metabolic homeostasis in mitochondria. May function as a nuclear-cytoplasmic shuttling protein that binds transcription factor RELA/NFKB3 in the nucleus and exports it to the cytoplasm. Suppresses p53-induced apoptosis by preventing nuclear localization of RELA.Involvement in disease:Defects in ETHE1 are a cause of ethylmalonic encephalopathy (EE) . EE is an autosomal recessive disorder characterized by neurodevelopmental delay and regression, recurrent petechiae, acrocyanosis, diarrhea, leading to death in the first decade of life. It is also associated with persistent lactic acidemia and ethylmalonic and methylsuccinic aciduria.

Gen-ID: 23474