酵母复合物 I 等效 NADH 脱氢酶可挽救 pink1 突变体。
The yeast complex I equivalent NADH dehydrogenase rescues pink1 mutants.
机构信息
VIB Center for Biology of Disease, Katholieke Universiteit Leuven, Leuven, Belgium.
出版信息
PLoS Genet. 2012 Jan;8(1):e1002456. doi: 10.1371/journal.pgen.1002456. Epub 2012 Jan 5.
Pink1 is a mitochondrial kinase involved in Parkinson's disease, and loss of Pink1 function affects mitochondrial morphology via a pathway involving Parkin and components of the mitochondrial remodeling machinery. Pink1 loss also affects the enzymatic activity of isolated Complex I of the electron transport chain (ETC); however, the primary defect in pink1 mutants is unclear. We tested the hypothesis that ETC deficiency is upstream of other pink1-associated phenotypes. We expressed Saccaromyces cerevisiae Ndi1p, an enzyme that bypasses ETC Complex I, or sea squirt Ciona intestinalis AOX, an enzyme that bypasses ETC Complex III and IV, in pink1 mutant Drosophila and find that expression of Ndi1p, but not of AOX, rescues pink1-associated defects. Likewise, loss of function of subunits that encode for Complex I-associated proteins displays many of the pink1-associated phenotypes, and these defects are rescued by Ndi1p expression. Conversely, expression of Ndi1p fails to rescue any of the parkin mutant phenotypes. Additionally, unlike pink1 mutants, fly parkin mutants do not show reduced enzymatic activity of Complex I, indicating that Ndi1p acts downstream or parallel to Pink1, but upstream or independent of Parkin. Furthermore, while increasing mitochondrial fission or decreasing mitochondrial fusion rescues mitochondrial morphological defects in pink1 mutants, these manipulations fail to significantly rescue the reduced enzymatic activity of Complex I, indicating that functional defects observed at the level of Complex I enzymatic activity in pink1 mutant mitochondria do not arise from morphological defects. Our data indicate a central role for Complex I dysfunction in pink1-associated defects, and our genetic analyses with heterologous ETC enzymes suggest that Ndi1p-dependent NADH dehydrogenase activity largely acts downstream of, or in parallel to, Pink1 but upstream of Parkin and mitochondrial remodeling.
Pink1 是一种与帕金森病相关的线粒体激酶,Pink1 功能的丧失会通过涉及 Parkin 和线粒体重塑机制成分的途径影响线粒体形态。Pink1 的缺失还会影响电子传递链(ETC)分离的复合物 I 的酶活性;然而,pink1 突变体的主要缺陷尚不清楚。我们检验了 ETC 缺陷是其他与 pink1 相关表型的上游因素这一假说。我们在 pink1 突变体果蝇中表达酿酒酵母 Ndi1p,一种绕过 ETC 复合物 I 的酶,或海鞘 Ciona intestinalis AOX,一种绕过 ETC 复合物 III 和 IV 的酶,发现 Ndi1p 的表达,而不是 AOX 的表达,可挽救与 pink1 相关的缺陷。同样,编码与复合物 I 相关蛋白的亚基的功能丧失显示出许多与 pink1 相关的表型,而这些缺陷可通过 Ndi1p 的表达来挽救。相反,Ndi1p 的表达不能挽救任何 parkin 突变体的表型。此外,与 pink1 突变体不同,果蝇 parkin 突变体没有表现出复合物 I 酶活性降低,表明 Ndi1p 作用于 Pink1 的下游或平行位置,但位于 Parkin 的上游或独立于 Parkin。此外,虽然增加线粒体分裂或减少线粒体融合可挽救 pink1 突变体中线粒体形态缺陷,但这些操作未能显著挽救复合物 I 酶活性的降低,表明在 pink1 突变体线粒体复合物 I 酶活性水平观察到的功能缺陷不是由形态缺陷引起的。我们的数据表明,复合物 I 功能障碍在与 pink1 相关的缺陷中起着核心作用,并且我们用异源 ETC 酶进行的遗传分析表明,Ndi1p 依赖性 NADH 脱氢酶活性主要作用于 Pink1 的下游或平行位置,但位于 Parkin 和线粒体重塑的上游。