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Mouse Anti-ETHE1 Recombinant Antibody (CBFYE-1333) (CBMAB-E1941-FY)

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Basic Information

Host Animal
Mouse
Clone
CBFYE-1333
Application
IHC
Immunogen
Human ETHE1.
Host Species
Mouse
Specificity
Human
Antibody Isotype
IgG2b
Clonality
Monoclonal Antibody
Application Notes
The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.
ApplicationNote
IHC1:100-1:200

Formulations & Storage [For reference only, actual COA shall prevail!]

Format
Liquid
Buffer
PBS
Preservative
Sodium azide
Concentration
Batch dependent
Purity
> 95% Purity determined by SDS-PAGE.
Storage
Store at +4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freezethaw cycles.
More Infomation

Target

Full Name
Ethe1, Persulfide Dioxygenase
Introduction
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.
Entrez Gene ID
23474
UniProt ID
O95571
Alternative Names
ETHE1, Persulfide Dioxygenase; Hepatoma Subtracted Clone One Protein; Ethylmalonic Encephalopathy 1; Sulfur Dioxygenase ETHE1; HSCO; Persulfide Dioxygenase ETHE1, Mitochondrial
Research Area
Sulfur dioxygenase that plays an essential role in hydrogen sulfide catabolism in the mitochondrial matrix. Hydrogen sulfide (H2S) 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 H2S 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).
Biological Process
Glutathione metabolic process Source: UniProtKB
Hydrogen sulfide metabolic process Source: UniProtKB
Cellular Location
Cytoplasm; Mitochondrion matrix; Nucleus
Involvement in disease
Ethylmalonic encephalopathy (EE):
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.

Sathe, G., Deepha, S., Gayathri, N., Nagappa, M., Sankaran, B. P., Taly, A. B., ... & Govindaraj, P. (2021). Ethylmalonic encephalopathy ETHE1 p. D165H mutation alters the mitochondrial function in human skeletal muscle proteome. Mitochondrion, 58, 64-71.

Al Balushi, A., Al Bakri, N., & Al Hashmi, N. (2021). A founder mutation in the ETHE1 gene and ethylmalonic encephalopathy in the Omani population. Journal of Biochemical and Clinical Genetics, 4(1), 51-51.

Ersoy, M., Tiranti, V., & Zeviani, M. (2020). Ethylmalonic encephalopathy: Clinical course and therapy response in an uncommon mild case with a severe ETHE1 mutation. Molecular Genetics and Metabolism Reports, 25, 100641.

Chen, X., Han, L., & Yao, H. (2020). Novel compound heterozygous variants of ETHE1 causing ethylmalonic encephalopathy in a Chinese patient: A case report. Frontiers in Genetics, 11, 341.

Witherspoon, M., Sandu, D., Lu, C., Wang, K., Edwards, R., Yeung, A., ... & Lipkin, S. (2019). ETHE1 overexpression promotes SIRT1 and PGC1α mediated aerobic glycolysis, oxidative phosphorylation, mitochondrial biogenesis and colorectal cancer. Oncotarget, 10(40), 4004.

Sahebekhtiari, N., Fernandez-Guerra, P., Nochi, Z., Carlsen, J., Bross, P., & Palmfeldt, J. (2019). Deficiency of the mitochondrial sulfide regulator ETHE1 disturbs cell growth, glutathione level and causes proteome alterations outside mitochondria. Biochimica et Biophysica Acta (BBA)-Molecular Basis of Disease, 1865(1), 126-135.

Grings, M., Seminotti, B., Karunanidhi, A., Ghaloul-Gonzalez, L., Mohsen, A. W., Wipf, P., ... & Leipnitz, G. (2019). ETHE1 and MOCS1 deficiencies: disruption of mitochondrial bioenergetics, dynamics, redox homeostasis and endoplasmic reticulum-mitochondria crosstalk in patient fibroblasts. Scientific reports, 9(1), 1-13.

Goudarzi, S., Babicz Jr, J. T., Kabil, O., Banerjee, R., & Solomon, E. I. (2018). Spectroscopic and electronic structure study of ETHE1: elucidating the factors influencing sulfur oxidation and oxygenation in mononuclear nonheme iron enzymes. Journal of the American Chemical Society, 140(44), 14887-14902.

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Please try the standard protocols which include: protocols, troubleshooting and guide.

Enzyme-linked Immunosorbent Assay (ELISA)
Flow Cytometry
Immunofluorescence (IF)
Immunohistochemistry (IHC)
Immunoprecipitation (IP)
Western Blot (WB)
Enzyme-Linked Immunospot (ELISpot)
Proteogenomics
Other Protocols

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Mouse Anti-ETHE1 Recombinant Antibody (CBFYE-1332) (CAT#: CBMAB-E1940-FY)

Isotype control

For research use only. Not intended for any clinical use.

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