ETHE1
ETHE1，即乙基丙二酸腦病1蛋白，也常被稱為線粒體硫雙加氧酶。這種蛋白主要存在于細胞的線粒體中，負責(zé)處理體內(nèi)代謝產(chǎn)生的有毒硫化物，將其轉(zhuǎn)化為無害的硫酸鹽，避免硫化物堆積損傷細胞。它的功能與線粒體能量生成密切相關(guān)——一旦活性不足，硫化物會干擾線粒體呼吸鏈運作，影響身體能量供應(yīng)。
與ETHE1相關(guān)的疾病最典型的是乙基丙二酸腦病，這是一種罕見的遺傳性疾病，患者多在嬰兒期發(fā)病，表現(xiàn)為發(fā)育遲緩、反復(fù)癲癇、嘔吐腹瀉及肢體無力等，嚴重時可致多器官衰竭。目前尚無針對性特效藥，臨床主要通過限制含硫食物攝入、補充維生素B12等緩解癥狀。
熱銷產(chǎn)品
Recombinant Human Protein ETHE1, mitochondrial (ETHE1) (CSB-EP007847HU)
驗證數(shù)據(jù)
(Tris-Glycine gel) Discontinuous SDS-PAGE (reduced) with 5% enrichment gel and 15% separation gel.
ETHE1 Antibody (CSB-PA007847HA01HU)
驗證數(shù)據(jù)
Western blot
All lanes: ETHE1 antibody at 8µg/ml
Lane 1: Mouse liver tissue
Lane 2: HepG2 whole cell lysate
Lane 3: Hela whole cell lysate
Secondary
Goat polyclonal to rabbit IgG at 1/10000 dilution
Predicted band size: 28 kDa
Observed band size: 28 kDa
Immunohistochemistry of paraffin-embedded human colon cancer using CSB-PA007847HA01HU at dilution of 1:100
ETHE1 Antibodies
ETHE1 for Homo sapiens (Human)
| 產(chǎn)品貨號 | 產(chǎn)品名稱 | 種屬反應(yīng)性 | 應(yīng)用類型 |
|---|---|---|---|
| CSB-PA703411 | ETHE1 Antibody | Human,Mouse | ELISA,WB,IHC |
| CSB-PA808765 | ETHE1 Antibody | Human,Mouse | ELISA,WB,IHC |
| CSB-PA007847HA01HU | ETHE1 Antibody | Human, Mouse | ELISA, WB, IHC |
ETHE1 Proteins
ETHE1 Proteins for Homo sapiens (Human)
| 產(chǎn)品貨號 | 產(chǎn)品名稱 | 來源 |
|---|---|---|
| CSB-YP007847HU CSB-BP007847HU CSB-MP007847HU CSB-EP007847HU-B |
Recombinant Human Protein ETHE1, mitochondrial (ETHE1) | Yeast Baculovirus Mammalian cell In Vivo Biotinylation in E.coli |
| CSB-EP007847HU | Recombinant Human Protein ETHE1, mitochondrial (ETHE1) | E.coli |
