|Application ||WB, FC, IHC-P, E|
|Calculated MW||27873 Da|
|Antigen Region||103-130 aa|
|Other Names||Persulfide dioxygenase ETHE1, mitochondrial, Ethylmalonic encephalopathy protein 1, Hepatoma subtracted clone one protein, Sulfur dioxygenase ETHE1, ETHE1, HSCO|
|Target/Specificity||This ETHE1 antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 103-130 amino acids from the Central region of human ETHE1.|
|Format||Purified polyclonal antibody supplied in PBS with 0.09% (W/V) sodium azide. This antibody is prepared by Saturated Ammonium Sulfate (SAS) precipitation followed by dialysis against PBS.|
|Storage||Maintain refrigerated at 2-8°C for up to 2 weeks. For long term storage store at -20°C in small aliquots to prevent freeze-thaw cycles.|
|Precautions||ETHE1 Antibody (Center) is for research use only and not for use in diagnostic or therapeutic procedures.|
|Function||Sulfur dioxygenase that plays an essential role in hydrogen sulfide catabolism in the mitochondrial matrix. Hydrogen sulfide (H(2)S) is first oxidized by SQRDL, giving rise to cysteine persulfide residues. ETHE1 consumes molecular oxygen to catalyze the oxidation of the persulfide, once it has been transferred to a thiophilic acceptor, such as glutathione (R-SSH). Plays an important role in metabolic homeostasis in mitochondria by metabolizing hydrogen sulfide and preventing the accumulation of supraphysiological H(2)S levels that have toxic effects, due to the inhibition of cytochrome c oxidase. First described as a protein that can shuttle between the nucleus and the cytoplasm and suppress p53-induced apoptosis by sequestering the transcription factor RELA/NFKB3 in the cytoplasm and preventing its accumulation in the nucleus (PubMed:12398897).|
|Cellular Location||Cytoplasm. Nucleus. Mitochondrion matrix. Note=According to PubMed:12398897, it is cytoplasmic and nuclear According to PubMed:14732903, it is found in the mitochondrial matrix|
|Tissue Location||Ubiquitously expressed.|
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Provided below are standard protocols that you may find useful for product applications.
ETHE1 is a sulfur dioxygenase that localizes within the mitochondrial matrix. The enzyme functions in sulfide catabolism. Mutations in its gene result in ethylmalonic encephalopathy.
Tiranti,V., Nat. Med. 15 (2), 200-205 (2009)
Mineri,R., J. Med. Genet. 45 (7), 473-478 (2008)
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