Gene Symbol | ETHE1 |
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Entrez Gene | 23474 |
Alt Symbol | HSCO, YF13H12 |
Species | Human |
Gene Type | protein-coding |
Description | ethylmalonic encephalopathy 1 |
Other Description | ethylmalonic encephalopathy protein 1|hepatoma subtracted clone one protein|persulfide dioxygenase ETHE1, mitochondrial|protein ETHE1, mitochondrial|sulfur dioxygenase ETHE1 |
Swissprots | Q96HR0 Q9H001 O95571 |
Accessions | AAG09063 EAW57195 EAW57196 EAW57197 O95571 AI879202 AK309880 AK315346 BAG37744 AM392804 CAL37682 BC008250 AAH08250 D83198 BAA34595 XM_005258687 XP_005258744 XM_005258688 XP_005258745 XM_011526685 XP_011524987 NM_014297 NP_055112 |
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). {ECO:0000269|PubMed:12398897, ECO:0000269|PubMed:14732903, ECO:0000269|PubMed:19136963, ECO:0000269|PubMed:23144459}. |
Subcellular 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 Specificity | Ubiquitously expressed. {ECO:0000269|PubMed:14732903}. |
Top Pathways | Sulfur metabolism |
ETHE1 (B-12) - sc-393869 from Santa Cruz Biotechnology
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ETHE1 (T-17) - sc-161565 from Santa Cruz Biotechnology
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ETHE1 (Y-13) - sc-161566 from Santa Cruz Biotechnology
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Anti-ETHE1 antibody - ab154041 from Abcam
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Anti-ETHE1 antibody - ab67528 from Abcam
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Anti-ETHE1 antibody - ab126166 from Abcam
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Anti-ETHE1 antibody - ab108138 from Abcam
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Anti-ETHE1 antibody [EPR11697] - ab174302 from Abcam
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Anti-ETHE1 antibody - C-terminal - ab135589 from Abcam
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Anti-ETHE1 Antibody - LS-C307437 from LifeSpan Bioscience
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Anti-ETHE1 Antibody (aa92-141) - LS-C117062 from LifeSpan Bioscience
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Anti-ETHE1 Antibody (aa180-209) IHC-plus⢠- LS-B10538 from LifeSpan Bioscience
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Anti-ETHE1 Antibody (aa103-130) - LS-C98379 from LifeSpan Bioscience
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ETHE1 Antibody - NBP2-16385 from Novus Biologicals
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ETHE1 Antibody - NBP1-81689 from Novus Biologicals
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ETHE1 Antibody - H00023474-B01P from Novus Biologicals
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ETHE1 Antibody - NBP1-81690 from Novus Biologicals
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ETHE1 Antibody - NBP1-81688 from Novus Biologicals
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ETHE1 Antibody - NBP2-16384 from Novus Biologicals
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ETHE1 Polyclonal Antibody - bs-7610R from Bioss
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Anti-ETHE1 (6-19) antibody produced in rabbit - SAB1101592 from Sigma-Aldrich
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Anti-ETHE1 (201-215) antibody produced in rabbit - SAB1101593 from Sigma-Aldrich
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Anti-ETHE1 antibody produced in mouse - SAB1400456 from Sigma-Aldrich
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Anti-ETHE1 antibody produced in rabbit - HPA028360 from Sigma-Aldrich
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Anti-ETHE1 antibody produced in rabbit - HPA029029 from Sigma-Aldrich
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