{"product_id":"abcam-ab251791","title":"Abcam, ab251791, Anti-ETHE1 antibody","description":"\u003cp\u003eSize: 100µL\u003cbr\u003e\nRabbit Polyclonal ETHE1 antibody. Suitable for IHC-P, WB, ICC\/IF and reacts with Human, Mouse, Rat samples. Cited in 1 publication. Immunogen corresponding to Recombinant Fragment Protein within Human ETHE1 aa 150-250.\u003cbr\u003e\nKey facts\u003cbr\u003e\nHost species:Rabbit,\u003cbr\u003e\nClonality:Polyclonal,\u003cbr\u003e\nIsotype:IgG,\u003cbr\u003e\nCarrier free:No,\u003cbr\u003e\nReacts with:Mouse, Rat, Human,\u003cbr\u003e\nApplications:ICC\/IF, WB, IHC-PSee reactivity dataSee the reactivity data table below for information on validated species and application combinations.,\u003cbr\u003e\nImmunogen:Recombinant Fragment Protein within Human ETHE1 aa 150-250. The exact immunogen used to generate this antibody is proprietary information.O95571\u003c\/p\u003e\n\n\u003cp\u003eProperties and Storage Information:\u003cbr\u003e\nForm-Liquid, Purification technique-Affinity purification Immunogen, Storage buffer-pH: 7.2Preservative: 0.02% Sodium azideConstituents: PBS, 40% Glycerol (glycerin, glycerine), Shipped at conditions-Blue Ice, Appropriate short-term storage duration-1-2 weeks, Appropriate short-term storage conditions-+4°C, Appropriate long-term storage conditions--20°C, Aliquoting information-Upon delivery aliquot, Storage information-Avoid freeze \/ thaw cycle\u003c\/p\u003e\n\n\u003cp\u003eSupplementary Information:\u003cbr\u003e\nThis supplementary information is collated from multiple sources and compiled automatically.\u003cbr\u003e\nETHE1 also known as persulfide dioxygenase plays an important role in the breakdown of sulfur-containing compounds. It catalyzes the conversion of persulfides to sulfite in the mitochondrial matrix. The protein has an approximate mass of 26 kDa and is strongly expressed in tissues with high metabolic activity such as the liver kidney and muscle. It plays an important role in maintaining cellular function and energy balance by efficiently processing by-products of cysteine metabolism.\u003cbr\u003e\nBiological function summary\u003cbr\u003e\nETHE1 assists in detoxifying potentially harmful sulfur compounds. It forms a part of a complex with sulfide:quinone oxidoreductase (SQR) and thiosulfate sulfurtransferase (TST) to facilitate efficient sulfur metabolism. The importance of ETHE1 lies in its ability to prevent the accumulation of toxic sulfide levels which can impair cellular respiration and energy production. The protein's role as a persulfide dioxygenase places it in a unique position as one of the body's defenses against sulfide toxicity.\u003cbr\u003e\nPathways\u003cbr\u003e\nETHE1 has significant relevance in the sulfide oxidation pathway and cellular respiration processes. Particularly it interacts with SQR in the mitochondrial matrix to convert hydrogen sulfide to thiosulfate and sulfite which then enter the citric acid cycle. This critical pathway is associated with energy production and efficient cellular function. The cooperation between ETHE1 and SQR ensures that toxic sulfide levels do not rise protecting mitochondrial integrity.\u003cbr\u003e\nDefects in ETHE1 result in ethylmalonic encephalopathy a severe metabolic disorder. The lack of functional ETHE1 leads to the accumulation of toxic levels of hydrogen sulfide which may disrupt mitochondrial function and energy production. This disorder highlights the interconnectedness of ETHE1 with SQR as impairments in this protein complex can exacerbate the pathological condition. Understanding the role of ETHE1 in this disease helps target therapies aimed at reducing sulfide toxicity.\u003c\/p\u003e","brand":"Abcam","offers":[{"title":"Default Title","offer_id":46854089801897,"sku":"ab251791","price":0.99,"currency_code":"USD","in_stock":true}],"url":"https:\/\/iright.com\/products\/abcam-ab251791","provider":"Iright","version":"1.0","type":"link"}