MMUT
MMUT，全稱為甲基丙二酰輔酶A變位酶，常見別名為MUT。該蛋白參與維生素B12依賴性代謝過程，能夠催化甲基丙二酰輔酶A異構(gòu)化為琥珀酰輔酶A，對(duì)于能量代謝和細(xì)胞功能至關(guān)重要。在人類中，MMUT蛋白的異常會(huì)導(dǎo)致甲基丙二酸血癥，這是一種罕見的遺傳代謝病，患者體內(nèi)甲基丙二酸水平升高，表現(xiàn)為多系統(tǒng)損害，尤其是腦損傷，死亡率較高。目前，治療主要依靠飲食控制、左卡尼汀和維生素B12補(bǔ)充，部分嚴(yán)重患者可能需要肝腎移植。近年來，基因治療成為研究熱點(diǎn)，通過腺病毒、慢病毒等載體將正常MMUT基因?qū)牖颊唧w內(nèi)，以期恢復(fù)正常的代謝功能。此外，針對(duì)MMUT蛋白的藥物研發(fā)也在進(jìn)行中，旨在直接干預(yù)異常代謝過程，為患者提供更有效的治療選擇。
熱銷產(chǎn)品
Recombinant Human Methylmalonyl-CoA mutase, mitochondrial (MMUT) (CSB-EP015243HU)
驗(yàn)證數(shù)據(jù)
(Tris-Glycine gel) Discontinuous SDS-PAGE (reduced) with 5% enrichment gel and 15% separation gel.
MMUT Recombinant Monoclonal Antibody (CSB-RA557759A0HU)
驗(yàn)證數(shù)據(jù)
Flow cytometric analysis of methylmalonyl-CoA mutase expression in HepG2 cells using methylmalonyl-CoA mutase antibody. Green, isotype control; red, methylmalonyl-CoA mutase.
Immunocytochemical staining of HepG2 cells with methylmalonyl-CoA mutase antibody. Nuclei were stained blue with DAPI; Methylmalonyl-CoA mutase was stained magenta with Alexa Fluor? 647. Images were taken using Leica stellaris 5. Protein abundance based on laser Intensity and smart gain: Very Low. Scale bar, 20 μm.
Western blotting analysis using methylmalonyl-CoA mutase antibody. Total cell lysates (30 μg) from various cell lines were loaded and separated by SDS-PAGE. The blot was incubated with methylmalonyl-CoA mutase antibody and HRP-conjugated goat anti-rabbit secondary antibody respectively.
MMUT Antibodies
MMUT for Homo sapiens (Human)
| 產(chǎn)品貨號(hào) | 產(chǎn)品名稱 | 種屬反應(yīng)性 | 應(yīng)用類型 |
|---|---|---|---|
| CSB-PA015243GA01HU | MUT Antibody | Human,Mouse,Rat | ELISA,WB,IHC |
| CSB-MA015243A0m | MMUT Monoclonal Antibody | Human, Mouse,Rat | ELISA,WB |
| CSB-RA015243MA1HU | MUT Recombinant Monoclonal Antibody | Human | ELISA, IHC, FC |
| CSB-RA014006A0HU | MUT Recombinant Monoclonal Antibody | Human | ELISA, WB, FC |
| CSB-RA194787A0HU | MMUT Recombinant Monoclonal Antibody | Human, Mouse, Rat | ELISA, WB, ICC |
| CSB-RA557759A0HU | MMUT Recombinant Monoclonal Antibody | Human, Mouse, Rat | ELISA, WB, FC, ICC |
MMUT for Mus musculus (Mouse)
| 產(chǎn)品貨號(hào) | 產(chǎn)品名稱 | 種屬反應(yīng)性 | 應(yīng)用類型 |
|---|---|---|---|
| CSB-PA015243YA01MO | Mut Antibody | Mouse | ELISA |
| CSB-PA015243YB01MO | Mut Antibody, HRP conjugated | Mouse | ELISA |
| CSB-PA015243YD01MO | Mut Antibody, Biotin conjugated | Mouse | ELISA |
MMUT Proteins
MMUT Proteins for Homo sapiens (Human)
| 產(chǎn)品貨號(hào) | 產(chǎn)品名稱 | 來源 |
|---|---|---|
| CSB-YP015243HU CSB-BP015243HU CSB-MP015243HU CSB-EP015243HU-B |
Recombinant Human Methylmalonyl-CoA mutase, mitochondrial (MMUT) | Yeast Baculovirus Mammalian cell In Vivo Biotinylation in E.coli |
| CSB-EP015243HU | Recombinant Human Methylmalonyl-CoA mutase, mitochondrial (MMUT) | E.coli |
MMUT Proteins for Mus musculus (Mouse)
| 產(chǎn)品貨號(hào) | 產(chǎn)品名稱 | 來源 |
|---|---|---|
| CSB-YP015243MO | Recombinant Mouse Methylmalonyl-CoA mutase, mitochondrial (Mmut) | Yeast |
| CSB-EP015243MO | Recombinant Mouse Methylmalonyl-CoA mutase, mitochondrial (Mutc) | E.coli |
| CSB-BP015243MO CSB-MP015243MO CSB-EP015243MO-B |
Recombinant Mouse Methylmalonyl-CoA mutase, mitochondrial (Mmut) | Baculovirus Mammalian cell In Vivo Biotinylation in E.coli |
MMUT Proteins for Sus scrofa (Pig)
| 產(chǎn)品貨號(hào) | 產(chǎn)品名稱 | 來源 |
|---|---|---|
| CSB-YP822640PI CSB-EP822640PI CSB-BP822640PI CSB-MP822640PI CSB-EP822640PI-B |
Recombinant Pig Methylmalonyl-CoA mutase, mitochondrial (MUT), partial | Yeast E.coli Baculovirus Mammalian cell In Vivo Biotinylation in E.coli |
MMUT Proteins for Bos taurus (Bovine)
| 產(chǎn)品貨號(hào) | 產(chǎn)品名稱 | 來源 |
|---|---|---|
| CSB-YP867072BO CSB-EP867072BO CSB-BP867072BO CSB-MP867072BO CSB-EP867072BO-B |
Recombinant Bovine Methylmalonyl-CoA mutase, mitochondrial (MUT), partial | Yeast E.coli Baculovirus Mammalian cell In Vivo Biotinylation in E.coli |
