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HPA029029

Sigma-Aldrich

Anti-ETHE1 antibody produced in rabbit

enhanced validation

Prestige Antibodies® Powered by Atlas Antibodies, affinity isolated antibody, buffered aqueous glycerol solution, ab3

Synonym(s):

Anti-FLJ10737, Anti-HSCO, Anti-YF13H12, Anti-ethylmalonic encephalopathy 1

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About This Item

UNSPSC Code:
12352203
Human Protein Atlas Number:
NACRES:
NA.41

biological source

rabbit

Quality Level

conjugate

unconjugated

antibody form

affinity isolated antibody

antibody product type

primary antibodies

clone

polyclonal

product line

Prestige Antibodies® Powered by Atlas Antibodies

form

buffered aqueous glycerol solution

species reactivity

mouse, human, rat

enhanced validation

orthogonal RNAseq
Learn more about Antibody Enhanced Validation

technique(s)

immunoblotting: 0.04-0.4 μg/mL
immunofluorescence: 0.25-2 μg/mL
immunohistochemistry: 1:1000-1:2500

immunogen sequence

RTDFQQGCAKTLYHSVHEKIFTLPGDCLIYPAHDYHGFTVSTVEEERTLNPRLTLSCEEFVKIMGNLNLPKPQQIDFAVPANMRCG

UniProt accession no.

shipped in

wet ice

storage temp.

−20°C

target post-translational modification

unmodified

Gene Information

human ... ETHE1(23474)

General description

ETHE1 (persulfide/sulfur dioxygenase) is a member of metallo-β-lactamase superfamily. It codes for a β-lactamase–like, iron-coordinating metalloprotein. It consists of a mononuclear iron in its active site. In eukaryotes, it is found within the mitochondrial matrix.

Immunogen

ethylmalonic encephalopathy 1 recombinant protein epitope signature tag (PrEST)

Application

Anti-ETHE1 antibody produced in rabbit has been used in western blotting.

Biochem/physiol Actions

ETHE1 (persulfide/sulfur dioxygenase) participates in the catabolism of sulfide that accumulates to toxic levels in ethylmalonic encephalopathy. ETHE1 can transform persulfides to sulfite in the mitochondrial sulfide oxidation pathway. Mutations in ETHE1 cause ethylmalonic encephalopathy.

Features and Benefits

Prestige Antibodies® are highly characterized and extensively validated antibodies with the added benefit of all available characterization data for each target being accessible via the Human Protein Atlas portal linked just below the product name at the top of this page. The uniqueness and low cross-reactivity of the Prestige Antibodies® to other proteins are due to a thorough selection of antigen regions, affinity purification, and stringent selection. Prestige antigen controls are available for every corresponding Prestige Antibody and can be found in the linkage section.

Every Prestige Antibody is tested in the following ways:
  • IHC tissue array of 44 normal human tissues and 20 of the most common cancer type tissues.
  • Protein array of 364 human recombinant protein fragments.

Linkage

Corresponding Antigen APREST77869

Physical form

Solution in phosphate-buffered saline, pH 7.2, containing 40% glycerol and 0.02% sodium azide.

Legal Information

Prestige Antibodies is a registered trademark of Merck KGaA, Darmstadt, Germany

Disclaimer

Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.

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Storage Class Code

10 - Combustible liquids

WGK

WGK 1

Flash Point(F)

Not applicable

Flash Point(C)

Not applicable


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Characterization of patient mutations in human persulfide dioxygenase (ETHE1) involved in H2S catabolism.
Kabil O and Banerjee R
The Journal of Biological Chemistry, 287(53), 44561-44567 (2012)
Spectroscopic studies on Arabidopsis ETHE1, a glyoxalase II-like protein.
Holdorf MM, et al.
Journal of Inorganic Biochemistry, 102(9), 1825-1830 (2008)
CoQ deficiency causes disruption of mitochondrial sulfide oxidation, a new pathomechanism associated with this syndrome.
EMBO Molecular Medicine, 9(1), 78-95 (2017)
Marta Luna-Sánchez et al.
EMBO molecular medicine, 9(1), 78-95 (2016-11-20)
Coenzyme Q (CoQ) is a key component of the mitochondrial respiratory chain, but it also has several other functions in the cellular metabolism. One of them is to function as an electron carrier in the reaction catalyzed by sulfide:quinone oxidoreductase
Pilar González-García et al.
Human molecular genetics, 29(19), 3296-3311 (2020-09-26)
Abnormalities of one carbon, glutathione and sulfide metabolisms have recently emerged as novel pathomechanisms in diseases with mitochondrial dysfunction. However, the mechanisms underlying these abnormalities are not clear. Also, we recently showed that sulfide oxidation is impaired in Coenzyme Q10

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