bims-mitpro Biomed News
on Mitochondrial proteostasis
Issue of 2026–03–22
two papers selected by
Andreas Kohler, Umeå University
  1. Transplantation of encapsulated mitochondria alleviates dysfunction in mitochondrial and Parkinson's disease models.
  2. Structures of respiratory supercomplexes and ATP synthase oligomers in mammalian mitochondrial inner membrane.
  1. Cell. 2026 Mar 18. pii: S0092-8674(26)00230-8. [Epub ahead of print]
    Transplantation of encapsulated mitochondria alleviates dysfunction in mitochondrial and Parkinson's disease models.
    Shiwei Du, Qi Long, Yanshuang Zhou, Jiangqin Fu, Hao Wu, Liang Yang, Yaohang Xie, Yingzhe Ding, Maolei Zhang, Jingyi Guo, Mengfei Wang, Jiajun Lin, Mingli Hu, Jian Zhang, Deyang Yao, Wei Li, Feixiang Bao, Ge Xiang, Yi Wu, Yile Huang, Haozhao Liang, Rui Wang, Heying Li, Baodan Chen, Chong Li, Junwei Wang, Jiwei Zhang, Dajiang Qin, Jianwei Sun, Yun Zhu, Fei Sun, Wuming Wang, Gang Lu, Wai-Yee Chan, Hui Zhao, Chenli Liu, Xingguo Liu.
      Mitochondrial transplantation holds significant potential for the treatment of mitochondrial diseases. However, how to efficiently deliver exogenous mitochondria to somatic cells or tissues remains unresolved. We present a mitochondrial transplantation approach to deliver mitochondria into the cells and tissues of mice and monkeys with high efficiency, based on encapsulating mitochondria with vesicles derived from the plasma membrane of erythrocytes. Treatment with encapsulated mitochondria complemented the loss, deletion, or mutation of mitochondrial DNA, thereby rescuing the associated bioenergetic and biochemical defects in patient-derived cells with mitochondrial disorders. Furthermore, mitochondrial capsules rescued the mitochondrial DNA depletion syndrome and Leigh syndrome in Dguok-/- and Ndufs4-/- mouse models, respectively. Moreover, in a mouse model of Parkinson's disease, mitochondrial capsules rescued neuron loss, improved motor skills, and restored mitochondrial function in the affected brain regions. Our study demonstrates the potential of this mitochondrial capsule as a treatment for mitochondrial disorders and proposes an "organelle therapy" strategy in regenerative medicine.
    Keywords:  Parkinson’s disease; degenerative disease; extracellular vesicle; mitochondria; mitochondrial diseases; mitochondrial transfer; mtDNA depletion syndrome; mtDNA mutation; organelle therapy
    DOI:  https://doi.org/10.1016/j.cell.2026.02.023
  2. Nat Commun. 2026 Mar 17.
    Structures of respiratory supercomplexes and ATP synthase oligomers in mammalian mitochondrial inner membrane.
    Atsuki Nakano, Takahiro Masuya, Shinsuke Akisada, Moe Ishikawa-Fukuda, Kaoru Mitsuoka, Hideto Miyoshi, Masatoshi Murai, Ken Yokoyama.
      Understanding the functional mechanisms of membrane protein complexes requires structural analysis within their native membrane environment. Here, we applied cryo-electron microscopy to determine the structures of FoF1 ATP synthase and respiratory supercomplexes (SCs) on sub-mitochondrial particles (SMPs) isolated from bovine heart mitochondria. Most FoF1 complexes were observed as dimers stabilized by the regulatory factor IF₁, and a tetrameric assembly comprising two FoF1-IF₁ dimers arranged linearly was also identified. This finding indicates that the tetrameric units of FoF1 are present in the mitochondrial inner membrane and contribute to shaping cristae tips in mammalian mitochondria. Fo domain maps resolve the e-subunit- c₈-ring interface and show no discrete density for a tightly bound lipid within the c₈-ring. In addition to the previously reported SCs compositions CI₁CIII₂CIV₁ and CI₁CIII₂CIV₂, our analysis identified an additional assembly with the composition CI₁CIII₂CIV₃, as well as a CI₂CIII₂CIV₆ mega-complex. This approach enables rapid structural determination of FoF1 ATP synthase and SCs from minimal membrane fractions, providing a foundation for elucidating the molecular basis of metabolic disorders and mitochondrial diseases at the level of higher-order architecture.
    DOI:  https://doi.org/10.1038/s41467-026-70578-x
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