bs-7610R

DATASHEET

Host: Rabbit

Target Protein: ETHE1

Immunogen Range: 191-254/254

Clonality: Polyclonal

Isotype: IgG

Entrez Gene: 23474

Source: KLH conjugated synthetic peptide derived from human E1

Purification: Purified by Protein A.

Storage Buffer: 0.01M TBS(pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.

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

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.

Size: 100ul

Concentration: 1ug/ul

Applications: ELISA(1:500-1000)
IHC-P(1:200-400)
IHC-F(1:100-500)
IF(IHC-P)(1:50-200)
IF(IHC-F)(1:50-200)
IF(ICC)(1:50-200)

Predicted Molecular Weight: 26

Predicted Cross Reactive Species: Human
Mouse
Dog
Cow
Sheep
Pig
Rabbit

For research use only. Not intended for diagnostic or therapeutic use.