Full Product Name
ETHE1 Antibody, FITC conjugated
Product Synonym Names
Persulfide dioxygenase ETHE1; mitochondrial (EC:1.13.11.182 Publications; ETHE1; HSCO; Ethylmalonic encephalopathy protein 1; Hepatoma subtracted clone one protein; Sulfur dioxygenase ETHE1
Product Gene Name
anti-ETHE1 antibody
[Similar Products]
Research Use Only
For Research Use Only. Not for use in diagnostic procedures.
3D Structure
ModBase 3D Structure for O95571
Species Reactivity
Human, Mouse
Purity/Purification
>95%, Protein G Purified
Storage Buffer
Preservative: 0.03% Proclin 300
Constituents: 50% Glycerol, 0.01M PBS, PH 7.4
Immunogen
Recombinant human Persulfide dioxygenase ETHE1, mitochondrial protein
Santa Cruz Alternative
Potential replacement for Santa Cruz Biotechnology antibody catalog# sc-161566
Preparation and Storage
Shipped at 4 degree C. Upon delivery aliquot and store at -20 degree C or -80 degree C. Avoid repeated freeze.
ISO Certification
Manufactured in an ISO 13485:2003 and EN ISO 13485:2012 Certified Laboratory.
Other Notes
Small volumes of anti-ETHE1 antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.
Related Product Information for
anti-ETHE1 antibody
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 (PubMed:12398897).
Applications Tested/Suitable for anti-ETHE1 antibody
ELISA (EIA)
NCBI/Uniprot data below describe general gene information for ETHE1. It may not necessarily be applicable to this product.
NCBI Accession #
NP_055112.2
[Other Products]
NCBI GenBank Nucleotide #
NM_014297.3
[Other Products]
UniProt Primary Accession #
O95571
[Other Products]
UniProt Secondary Accession #
Q96HR0; Q9H001[Other Products]
UniProt Related Accession #
O95571[Other Products]
Molecular Weight
27,873 Da
NCBI Official Full Name
persulfide dioxygenase ETHE1, mitochondrial
NCBI Official Synonym Full Names
ethylmalonic encephalopathy 1
NCBI Official Symbol
ETHE1 [Similar Products]
NCBI Official Synonym Symbols
HSCO; YF13H12
[Similar Products]
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 [Similar Products]
UniProt Synonym Gene Names
HSCO [Similar Products]
UniProt Entry Name
ETHE1_HUMAN
NCBI Summary for ETHE1
This gene encodes a sulfur dioxygenase that localizes within the mitochondrial matrix. The enzyme functions in sulfide catabolism. Mutations in this gene result in ethylmalonic encephalopathy.[provided by RefSeq, May 2009]
UniProt Comments for ETHE1
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.
Protein type: Hydrolase; Mitochondrial; 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; sulfur amino acid catabolic process; sulfur amino acid metabolic process
Disease: Encephalopathy, Ethylmalonic
Research Articles on ETHE1
1. ETHE1 R163W/R163Q mutations are associated with Ethylmalonic encephalopathy.
Precautions
All of MyBioSource's Products are for scientific laboratory research purposes and are not for diagnostic, therapeutics, prophylactic or in vivo use. Through your purchase, you expressly represent and warrant to MyBioSource that you will properly test and use any Products purchased from MyBioSource in accordance with industry standards. MyBioSource and its authorized distributors reserve the right to refuse to process any order where we reasonably believe that the intended use will fall outside of our acceptable guidelines.
Disclaimer
While every efforts were made to ensure the accuracy of the information provided in this datasheet, MyBioSource will not be liable for any omissions or errors contained herein. MyBioSource reserves the right to make changes to this datasheet at any time without prior notice.
It is the responsibility of the customer to report product performance issues to MyBioSource within 30 days of receipt of the product. Please visit our Terms & Conditions page for more information.
