ETHE1 Polyclonal Antibody, FITC Conjugated
Product Specifications
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.
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.
Gene ID
23474
Cellular Locus
Cytoplasm, Nucleus
Host
Rabbit
Immunogen
191-254/254
Target
ETHE1
Clonality
Polyclonal
Isotype
IgG
Conjugation
FITC
Source
KLH conjugated synthetic peptide derived from human E1
Applications
IF (IHC-P), IF (IHC-F), IF (ICC)
Purification
Purified by Protein A.
Excitation Emission
494nm/518nm
Concentration
1µg/µl
Dilution
IF (IHC-P) (1:50-200), IF (IHC-F) (1:50-200), IF (ICC) (1:50-200)
Buffer
Aqueous buffered solution containing 0.01M TBS (pH 7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
Modification
Unmodified
Storage Conditions
Store at -20°C. Aliquot into multiple vials to avoid repeated freeze-thaw cycles.
Gene ID URL
23474
Predicted Cross Reactivity
Human, Mouse, Dog, Cow, Sheep, Pig, Rabbit
Curated Selection
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