ETHE1 Antibody, FITC conjugated (PACO26567)
- SKU:
- PACO26567
- Product type:
- Antibody
- Reactivity:
- Human
- Host Species:
- Rabbit
- Isotype:
- IgG
- Application:
- ELISA
- Antibody type:
- Polyclonal
- Conjugation:
- FITC
Frequently bought together:
Description
抗体名: | ETHE1 Antibody, FITC conjugated (PACO26567) |
抗体コード: | PACO26567 |
サイズ: | 50ug |
宿主種: | Rabbit |
申し込み: | ELISA |
推奨される希釈: | |
反応性: | Human |
免疫原: | Recombinant Human Persulfide dioxygenase ETHE1, mitochondrial protein (8-254AA) |
憲法: | Liquid |
ストレージバッファ: | Preservative: 0.03% Proclin 300 Constituents: 50% Glycerol, 0.01M PBS, PH 7.4 |
精製方法: | >95%, Protein G purified |
抗体のクローン性: | Polyclonal |
アイソタイプ: | IgG |
Conjugate: | FITC |
バックグラウンド: | 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). |
シノニム: | Persulfide dioxygenase ETHE1, mitochondrial (EC 1.13.11.18) (Ethylmalonic encephalopathy protein 1) (Hepatoma subtracted clone one protein) (Sulfur dioxygenase ETHE1), ETHE1, HSCO |
UniProt Protein Function: | ETHE1: 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. 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. Belongs to the metallo-beta-lactamase superfamily. Glyoxalase II family. |
UniProt Protein Details: | Protein type:Mitochondrial; Hydrolase; EC 1.13.11.18 Chromosomal Location of Human Ortholog: 19q13.31 Cellular Component: cytoplasm; mitochondrial matrix; mitochondrion; nucleoplasm Molecular Function:iron ion binding; sulfur dioxygenase activity Biological Process: glutathione metabolic process Disease: Encephalopathy, Ethylmalonic |
NCBI Summary: | This gene encodes a member of the metallo beta-lactamase family of iron-containing proteins involved in the mitochondrial sulfide oxidation pathway. The encoded protein catalyzes the oxidation of a persulfide substrate to sulfite. Certain mutations in this gene cause ethylmalonic encephalopathy, an infantile metabolic disorder affecting the brain, gastrointestinal tract and peripheral vessels. Alternative splicing results in multiple transcript variants encoding different isoforms. [provided by RefSeq, Mar 2016] |
UniProt Code: | O95571 |
NCBI GenInfo Identifier: | 73919341 |
NCBI Gene ID: | 23474 |
NCBI Accession: | O95571.2 |
UniProt Secondary Accession: | O95571,Q96HR0, Q9H001, |
UniProt Related Accession: | O95571 |
Molecular Weight: | 27,873 Da |
NCBI Full Name: | Persulfide dioxygenase ETHE1, mitochondrial |
NCBI Synonym Full Names: | ETHE1, persulfide dioxygenase |
NCBI Official Symbol: | ETHE1 |
NCBI Official Synonym Symbols: | HSCO; YF13H12 |
NCBI Protein Information: | persulfide dioxygenase ETHE1, mitochondrial |
UniProt Protein Name: | Persulfide dioxygenase ETHE1, mitochondrial |
UniProt Synonym Protein Names: | Ethylmalonic encephalopathy protein 1; Hepatoma subtracted clone one protein; Sulfur dioxygenase ETHE1 |
Protein Family: | Persulfide dioxygenase |
UniProt Gene Name: | ETHE1 |
UniProt Entry Name: | ETHE1_HUMAN |