ETHE1 anticorps (AA 191-254) (AbBy Fluor® 555)

N° du produit ABIN895783

Cet anticorps Lapin Polyclonal détecte spécifiquement ETHE1 dans IF (cc) et IF (p). Il présente une réactivité envers Humain.

Aperçu rapide pour ETHE1 anticorps (AA 191-254) (AbBy Fluor® 555) (ABIN895783)

Antigène: ETHE1 (Ethylmalonic Encephalopathy 1 (ETHE1))

Reactivité: Humain

Hôte: Lapin

Clonalité: Polyclonal

Conjugué: Cet anticorp ETHE1 est conjugé à/à la AbBy Fluor® 555

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

Détail du produit anti-ETHE1 anticorps (AbBy Fluor® 555)

Épitope: AA 191-254

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

Purification: Purified by Protein A.

Immunogène: KLH conjugated synthetic peptide derived from human E1

Isotype: IgG

Information d'application

Indications d'application: IF(IHC-P) 1:50-200
IF(IHC-F) 1:50-200
IF(ICC) 1:50-200

Restrictions: For Research Use only

Stockage

Format: Liquid

Concentration: 1 μg/μL

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

Stockage commentaire: Store at -20°C. Aliquot into multiple vials to avoid repeated freeze-thaw cycles.

Date de péremption: 12 months

Détails sur ETHE1

Antigène: ETHE1 (Ethylmalonic Encephalopathy 1 (ETHE1))

Autre désignation: ETHE1

Sujet:

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.

ID gène: 23474