ETHE1 Antibody

Code CSB-PA007847HA01HU
Size US$166
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Image
  • Western blot
    All lanes: ETHE1 antibody at 8µg/ml
    Lane 1: Mouse liver tissue
    Lane 2: HepG2 whole cell lysate
    Lane 3: Hela whole cell lysate
    Secondary
    Goat polyclonal to rabbit IgG at 1/10000 dilution
    Predicted band size: 28 kDa
    Observed band size: 28 kDa

  • Immunohistochemistry of paraffin-embedded human colon cancer using CSB-PA007847HA01HU at dilution of 1:100

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Product Details

Full Product Name
Rabbit anti-Homo sapiens (Human) ETHE1 Polyclonal antibody
Uniprot No.
Target Names
ETHE1
Alternative Names
Ethe1 antibody; ETHE1 protein; mitochondrial precursor antibody; ETHE1_HUMAN antibody; ethylmalonic encephalopathy 1 antibody; Ethylmalonic encephalopathy protein 1 antibody; hepatoma subtracted clone one antibody; Hepatoma subtracted clone one protein antibody; HSCO antibody; mitochondrial antibody; Protein ETHE1 antibody; Protein ETHE1 mitochondrial antibody; YF13H12 antibody
Raised in
Rabbit
Species Reactivity
Human, Mouse
Immunogen
Recombinant Human Persulfide dioxygenase ETHE1, mitochondrial protein (8-254AA)
Immunogen Species
Homo sapiens (Human)
Conjugate
Non-conjugated

The ETHE1 Antibody (Product code: CSB-PA007847HA01HU) is Non-conjugated. For ETHE1 Antibody with conjugates, please check the following table.

Available Conjugates
Conjugate Product Code Product Name Application
HRP CSB-PA007847HB01HU ETHE1 Antibody, HRP conjugated ELISA
FITC CSB-PA007847HC01HU ETHE1 Antibody, FITC conjugated
Biotin CSB-PA007847HD01HU ETHE1 Antibody, Biotin conjugated ELISA
Clonality
Polyclonal
Isotype
IgG
Purification Method
>95%, Protein G purified
Concentration
It differs from different batches. Please contact us to confirm it.
Buffer
Preservative: 0.03% Proclin 300
Constituents: 50% Glycerol, 0.01M PBS, PH 7.4
Form
Liquid
Tested Applications
ELISA, WB, IHC
Recommended Dilution
Application Recommended Dilution
WB 1:500-1:2000
IHC 1:20-1:200
Troubleshooting and FAQs
Storage
Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
Lead Time
Basically, we can dispatch the products out in 1-3 working days after receiving your orders. Delivery time maybe differs from different purchasing way or location, please kindly consult your local distributors for specific delivery time.

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Target Background

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.
Gene References into Functions
  1. observations indicate the severe impact of ETHE1 deficiency on cellular physiology and redox state PMID: 27074420
  2. ETHE1 is a major enzyme regulating endogenous glutathione persulfide /GS-(S)n-H and that its activity is controlled by polysulfidation of the Cys247 residue. PMID: 27742479
  3. ETHE1 R163W/R163Q mutations are associated with Ethylmalonic encephalopathy. PMID: 25198162
  4. Case Report: metabolic disturbances in 15-month-old male presenting with typical ethylmalonic encephalopathy associated with a homozygous ETHE1 mutation. PMID: 20978941
  5. T152I mutation of ETHE1 results in a 3-fold lower activity. PMID: 23144459
  6. role of the ETHE1 gene product in mitochondrial homeostasis and energy metabolism PMID: 14732903
  7. Mutations of ETHE1 were detected in all the typical ethylmalonic encephalopathy patients analysed, but no ETHE1 mutations were identified in patients presenting with early onset progressive encephalopathy with ethylmalonic aciduria. PMID: 16183799
  8. structural comparison of human ETHE1 and At1g53580 from Arabidopsis thaliana PMID: 16929096
  9. 14 patients with EE were investigated for mutations in the ETHE1 gene. Of the 14 patients, 5 were found to carry novel mutations. PMID: 18593870
  10. ETHE1 is a mitochondrial sulfur dioxygenase involved in catabolism of sulfide that accumulates to toxic levels in ethylmalonic encephalopathy. PMID: 19136963

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Involvement in disease
Ethylmalonic encephalopathy (EE)
Subcellular Location
Cytoplasm. Nucleus. Mitochondrion matrix.
Protein Families
Metallo-beta-lactamase superfamily, Glyoxalase II family
Tissue Specificity
Ubiquitously expressed.
Database Links

HGNC: 23287

OMIM: 602473

KEGG: hsa:23474

STRING: 9606.ENSP00000292147

UniGene: Hs.7486

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