ETHE1 Antibody

Datasheet

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
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Target Background

Product Details

Full Product Name

Rabbit anti-Homo sapiens (Human) ETHE1 Polyclonal antibody

Uniprot No.

O95571

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.

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

Protocols

ELISA Protocol
Western Blotting(WB) Protocol
Immunohistochemistry (IHC) Protocol

Troubleshooting and FAQs

Antibody 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.

Usage

For Research Use Only. Not for use in diagnostic or therapeutic procedures.

Customer Reviews and Q&A

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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

observations indicate the severe impact of ETHE1 deficiency on cellular physiology and redox state PMID: 27074420
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
ETHE1 R163W/R163Q mutations are associated with Ethylmalonic encephalopathy. PMID: 25198162
Case Report: metabolic disturbances in 15-month-old male presenting with typical ethylmalonic encephalopathy associated with a homozygous ETHE1 mutation. PMID: 20978941
T152I mutation of ETHE1 results in a 3-fold lower activity. PMID: 23144459
role of the ETHE1 gene product in mitochondrial homeostasis and energy metabolism PMID: 14732903
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
structural comparison of human ETHE1 and At1g53580 from Arabidopsis thaliana PMID: 16929096
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
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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Antibody FAQs What Is an Antibody? How to Choose an Antibody for Scientific Research? The difference between primary antibody and secondary antibod The Meaning of Irregular Antibody Screening Tag Antibodies, Your Good Partner in Protein Research How to Choose the Loading Control Antibodies?

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301-363-4651 (Available 9 a.m. to 5 p.m. CST from Monday to Friday)

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