Mouse Ethylmalonic Encephalopathy 1 (ETHE1) ELISA Kit-Sandwich

ETHE1 (Ethylmalonic Encephalopathy 1) is a member of the iron-containing metallo-β-lactamase family of proteins, primarily situated in the mitochondrial matrix, and plays a crucial role in hydrogen sulfide (H₂S) metabolism. In mice, research into ETHE1's function and associated disease mechanisms highlights its importance in maintaining cellular energy metabolism, redox balance, and the regulation of protein translation. The absence of ETHE1 results in markedly elevated hydrogen sulfide levels, which trigger a cascade of metabolic disorders and toxic effects. For example, mice lacking ETHE1 exhibit reduced activity of mitochondrial cytochrome c oxidase (COX), particularly in the colon, muscle, and brain tissues. In liver tissue of ETHE1-deficient mice, the expression of several redox-related proteins, such as dehydrogenases and cytochrome P450 family members, decreases, while glycolysis-related enzyme expression increases, suggesting that ETHE1 deficiency may alter cell function by remodeling metabolic pathways.