Wong Yvette C, Kim Soojin, Cisneros Jasmine, Molakal Catherine G, Song Pingping, Lubbe Steven J, Krainc Dimitri
Department of Neurology, Northwestern University Feinberg School of Medicine, Chicago, IL.
Simpson Querrey Center for Neurogenetics, Northwestern University Feinberg School of Medicine, Chicago, IL.
J Cell Biol. 2022 Oct 3;221(10). doi: 10.1083/jcb.202206140. Epub 2022 Sep 1.
Lysosomes are highly dynamic organelles implicated in multiple diseases. Using live super-resolution microscopy, we found that lysosomal tethering events rarely undergo lysosomal fusion, but rather untether over time to reorganize the lysosomal network. Inter-lysosomal untethering events are driven by a mitochondrial Mid51/Fis1 complex that undergoes coupled oligomerization on the outer mitochondrial membrane. Importantly, Fis1 oligomerization mediates TBC1D15 (Rab7-GAP) mitochondrial recruitment to drive inter-lysosomal untethering via Rab7 GTP hydrolysis. Moreover, inhibiting Fis1 oligomerization by either mutant Fis1 or a Mid51 oligomerization mutant potentially associated with Parkinson's disease prevents lysosomal untethering events, resulting in misregulated lysosomal network dynamics. In contrast, dominant optic atrophy-linked mutant Mid51, which does not inhibit Mid51/Fis1 coupled oligomerization, does not disrupt downstream lysosomal dynamics. As Fis1 conversely also regulates Mid51 oligomerization, our work further highlights an oligomeric Mid51/Fis1 mitochondrial complex that mechanistically couples together both Drp1 and Rab7 GTP hydrolysis machinery at mitochondria-lysosome contact sites. These findings have significant implications for organelle networks in cellular homeostasis and human disease.
溶酶体是与多种疾病相关的高度动态的细胞器。利用实时超分辨率显微镜,我们发现溶酶体拴系事件很少发生溶酶体融合,而是随着时间的推移解开拴系以重组溶酶体网络。溶酶体间的解开拴系事件由线粒体外膜上发生偶联寡聚化的线粒体Mid51/Fis1复合体驱动。重要的是,Fis1寡聚化介导TBC1D15(Rab7-GAP)向线粒体募集,通过Rab7 GTP水解驱动溶酶体间的解开拴系。此外,通过突变型Fis1或可能与帕金森病相关的Mid51寡聚化突变体抑制Fis1寡聚化可防止溶酶体解开拴系事件,导致溶酶体网络动力学失调。相比之下,与显性视神经萎缩相关的突变型Mid51不抑制Mid51/Fis1偶联寡聚化,不会破坏下游溶酶体动力学。由于Fis1反过来也调节Mid51寡聚化,我们的工作进一步突出了一种寡聚化的Mid51/Fis1线粒体复合体,该复合体在机制上将Drp1和Rab7 GTP水解机制在线粒体-溶酶体接触位点耦合在一起。这些发现对细胞稳态和人类疾病中的细胞器网络具有重要意义。