产品详情
简单介绍：
Anti-ETHE1 Antibody
详情介绍：
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: |
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| Synonyms: | |
| Information: | Target information shown above is from the UniProt Consortium. |
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