Cell Biology Antibodies 1
Anti-ETHE1 Antibody (CAB10142)
- SKU:
- CAB10142
- Product Type:
- Antibody
- Reactivity:
- Human
- Reactivity:
- Mouse
- Reactivity:
- Rat
- Host Species:
- Rabbit
- Isotype:
- IgG
- Antibody Type:
- Polyclonal Antibody
- Research Area:
- Cell Biology
Description
Antibody Name: | Anti-ETHE1 Antibody |
Antibody SKU: | CAB10142 |
Antibody Size: | 20uL, 50uL, 100uL |
Application: | WB IHC IF |
Reactivity: | Human, Mouse, Rat |
Host Species: | Rabbit |
Immunogen: | Recombinant fusion protein containing a sequence corresponding to amino acids 8-245 of human ETHE1 (NP_055112.2). |
Application: | WB IHC IF |
Recommended Dilution: | WB 1:200 - 1:1000 IHC 1:50 - 1:200 IF 1:50 - 1:200 |
Reactivity: | Human, Mouse, Rat |
Positive Samples: | SW480, Mouse liver, Mouse kidney, Rat liver |
Immunogen: | Recombinant fusion protein containing a sequence corresponding to amino acids 8-245 of human ETHE1 (NP_055112.2). |
Purification Method: | Affinity purification |
Storage Buffer: | Store at -20'C. Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3. |
Isotype: | IgG |
Sequence: | VARR QLSQ RGGS GAPI LLRQ MFEP VSCT FTYL LGDR ESRE AVLI DPVL ETAP RDAQ LIKE LGLR LLYA VNTH CHAD HITG SGLL RSLL PGCQ SVIS RLSG AQAD LHIE DGDS IRFG RFAL ETRA SPGH TPGC VTFV LNDH SMAF TGDA LLIR GCGR TDFQ QGCA KTLY HSVH EKIF TLPG DCLI YPAH DYHG FTVS TVEE ERTL NPRL TLSC EEFV KIMG NLNL PKPQ QIDF AVPA NM |
Gene ID: | 23474 |
Uniprot: | O95571 |
Cellular Location: | Cytoplasm, Mitochondrion matrix, Nucleus |
Calculated MW: | 27kDa |
Observed MW: | 28kDa |
Synonyms: | ETHE1, HSCO, YF13H12 |
Background: | 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. |
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 |