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Antibodies
Anti-ETHE1 Antibody
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产品名称:
Anti-ETHE1 Antibody
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简单介绍
Anti-ETHE1
Antibody
Anti-ETHE1 Antibody
的详细介绍
Overview
Name:
Anti-ETHE1
Antibody
See all ETHE1 primary antibodies
Description:
Rabbit polyclonal antibody to ETHE1
Specificity:
The antibody detects endogenous levels of total ETHE1 protein.
Applications:
WB, IHC
Reactivity:
Human, Mouse
Immunogen:
Fusion protein corresponding to residues near the N terminal of human ethylmalonic encephalopathy 1
Host:
Rabbit
Clonality:
Polyclonal
Conjugate:
Unconjugated
Purification:
Antigen affinity purification.
Concentration:
1.6mg / ml
Formulation:
Rabbit IgG in pH7.3 PBS, 0.05% NaN3, 50% Glycerol.
Storage:
Store at -20?C
Target
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 (PubMed:12398897).
Tissue Specificity:
Ubiquitously expressed.
Involvement in Disease:
Ethylmalonic encephalopathy: 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.
Sequence Similarities:
Belongs to the metallo-beta-lactamase superfamily. Glyoxalase II family.
Cellular Location:
Cytoplasm. Nucleus. Mitochondrion matrix.
Database Links:
Entrez Gene: 23474?Human
Entrez Gene: 66071?Mouse
Omim: 608451?Human
SwissProt: O95571?Human
SwissProt: Q9DCM0?Mouse
Unigene: 7486?Human
Unigene: 29553?Mouse
Synonyms:
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
Information:
Target information shown above is from the UniProt Consortium.
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