YRP07615-01 [Polyclonal Antibody]
ETHE1 Rabbit Polyclonal Antibody
www.yhsbio.com
market@yhsbio.com
support@yhsbio.com
+86-21-54651191
Room 703,Building 6,333# Guiping
Rd.,Xuhui District,Shanghai,China
DATASHEET
Species:   Rabbit
Applications:   WB IHC IF
Immunogen Range:   KLH conjugated synthetic peptide derived from human E1
Clonality:   Polyclonal Antibody
Isotype:   IgG
GENE ID:   23474
Swiss Prot:  
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.
Purification:   Purified by Protein A.
Storage:   Aqueous buffered solution containing 100ug/ml BSA, 50% glycerol and 0.09% sodium azide. Store at -20℃ for 12 months
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.
Caculated MW:   /
Observed MW:   Refer to Figures
Applications:   WB 1:100-1:1000
IHC 1:100-1:500
IF 1:50-1:200
Reacitivity:   Human, Mouse
For research use only. Not intended for diagnostic or therapeutic use!
Additional information