近日,,神經(jīng)學(xué)家Patrik Verstreken等人成功解開(kāi)導(dǎo)致帕金森氏服用維生素K2的遺傳缺陷。他的發(fā)現(xiàn)為帕金森氏癥患者帶來(lái)了希望,。這項(xiàng)研究刊登在權(quán)威雜志Science上,。
Patrik Verstreken說(shuō)我們的研究顯示,服用維生素K2可能幫助帕金森氏癥患者,。然而,,需要開(kāi)展更多的研究工作以更好地理解其中機(jī)制。
這種神經(jīng)退行性疾病的確切原因尚不清楚,。然而近年來(lái),,科學(xué)家已經(jīng)能夠確定一些遺傳缺陷(突變)與帕金森氏癥有關(guān),其中包括所謂的PINK1基因和Parkin基因突變,,這些都導(dǎo)致線(xiàn)粒體活性降低,。通過(guò)研究這些基因突變,,科學(xué)家們希望能夠解開(kāi)疾病過(guò)程的機(jī)制。
癱瘓的果蠅
果蠅在實(shí)驗(yàn)室實(shí)驗(yàn)研究中經(jīng)常使用,,因?yàn)樗麄兊纳芷诙?、繁殖周期短等特點(diǎn)。通過(guò)轉(zhuǎn)基因改造的果蠅,,科學(xué)家可以研究某些基因和蛋白質(zhì)的功能,。Patrik Verstreken和他的團(tuán)隊(duì)使用PINK1基因遺傳缺陷果蠅或Parkin基因遺傳缺陷果蠅。他們發(fā)現(xiàn)PINK1基因突變或Parkin基因突變后會(huì)導(dǎo)致蒼蠅失去了他們的飛行能力,。
經(jīng)仔細(xì)檢查,,他們發(fā)現(xiàn)這些線(xiàn)粒體有缺陷的果蠅,就像帕金森氏癥患者,。當(dāng)果蠅給予維生素K2,,其線(xiàn)粒體的能源生產(chǎn)得到恢復(fù),昆蟲(chóng)的飛行能力也改善,。
結(jié)論
維生素K2在有缺陷的線(xiàn)粒體能量生產(chǎn)中發(fā)揮作用,。因?yàn)镻INK1基因或Parkin突變的帕金森氏癥患者中發(fā)現(xiàn)有缺陷的線(xiàn)粒體,維生素K2可能為治療帕金森氏帶來(lái)了新的希望,。(生物谷:Bioon.com)
doi:10.1126/science.1218632
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Vitamin K2 Is a Mitochondrial Electron Carrier That Rescues Pink1 Deficiency
Melissa Vos, Giovanni Esposito, Janaka N. Edirisinghe, Sven Vilain, Dominik M. Haddad1, Jan R. Slabbaert, Stefanie Van Meensel, et al.
Human UBIAD1 localizes to mitochondria and converts vitamin K1 to vitamin K2. Vitamin K2 is best known as a cofactor in blood coagulation, but in bacteria it is a membrane-bound electron carrier. Whether vitamin K2 exerts a similar carrier function in eukaryotic cells is unknown. We identified Drosophila UBIAD1/Heix as a modifier of pink1, a gene mutated in Parkinson’s disease that affects mitochondrial function. Here, we found that vitamin K2 was necessary and sufficient to transfer electrons in Drosophila mitochondria. Heix mutants showed severe mitochondrial defects that were rescued by vitamin K2, and, similar to ubiquinone, vitamin K2 transferred electrons in Drosophila mitochondria, resulting in more efficient adenosine triphosphate (ATP) production. Thus, mitochondrial dysfunction was rescued by vitamin K2 that serves as a mitochondrial electron carrier, helping to maintain normal ATP production.