Anti-ETHE1 Rabbit Polyclonal Antibody (Alexa Fluor® 750)

Lieferant: Bioss
BS-7610R-A750
BOSSBS-7610R-A750EA 893 EUR
BOSSBS-7610R-A750
Anti-ETHE1 Rabbit Polyclonal Antibody (Alexa Fluor® 750)
Antikörper
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 localisation of RELA.Involvement in disease:Defects in ETHE1 are a cause of ethylmalonic encephalopathy (EE) . EE is an autosomal recessive disorder characterised 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.

Type: Primary
Antigen: ETHE1
Clonality: Polyclonal
Clone:
Conjugation: ALEXA FLUOR® 750
Public Immunogen Range: 191-254/254
Host: Rabbit
Isotype: IgG
Reactivity:
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