+Search query
-Structure paper
| Title | Structural remodeling of the mitochondrial protein biogenesis machinery under proteostatic stress. |
|---|---|
| Journal, issue, pages | Sci Adv, Vol. 12, Issue 10, Page eaed3579, Year 2026 |
| Publish date | Mar 6, 2026 |
Authors | Kenneth Ehses / Jorge P López-Alonso / Odetta Antico / Yannik Lang / Till Rudack / Abdussalam Azem / Miratul M K Muqit / Iban Ubarretxena-Belandia / Rubén Fernández-Busnadiego / ![]() |
| PubMed Abstract | Cells have evolved organelle-specific responses to maintain protein homeostasis (proteostasis). During proteostatic stress, mitochondria down-regulate translation and enhance protein folding, yet the ...Cells have evolved organelle-specific responses to maintain protein homeostasis (proteostasis). During proteostatic stress, mitochondria down-regulate translation and enhance protein folding, yet the underlying mechanisms remain poorly defined. Here, we used cryo-electron tomography to observe the structural consequences of mitochondrial proteostatic stress within human cells. We detected protein aggregates within the mitochondrial matrix, accompanied by a marked remodeling of cristae architecture. Concomitantly, the number of mitochondrial ribosome complexes was significantly reduced. Mitochondrial Hsp60 (mHsp60), a key protein folding machine, underwent major conformational changes to favor complexes with its co-chaperone mHsp10. We visualized the interactions of mHsp60 with native substrate proteins and determined in vitro mHsp60 cryo-electron microscopy structures enabling nucleotide state assignment of the in situ structures. These data converge on a model of the mHsp60 functional cycle and its essential role in mitochondrial proteostasis. More broadly, our findings reveal structural mechanisms governing mitochondrial protein biosynthesis and their remodeling under proteostatic stress. |
External links | Sci Adv / PubMed:41779846 / PubMed Central |
| Methods | EM (subtomogram averaging) |
| Resolution | 7.76 - 22.89 Å |
| Structure data | ![]() EMDB-55203: Subtomogram average of the 39S mitochondrial ribosome from HeLa ![]() EMDB-55204: Subtomogram average of the 55S mitochondrial ribosome from HeLa ![]() EMDB-55205: Subtomogram average of the mtHsp60 singlering complex from HeLa ![]() EMDB-55206: Subtomogram average of the mtHsp60:mtHsp10 half-football complex from HeLa ![]() EMDB-55207: Subtomogram average of the mtHsp60:mtHsp10 Football complex from HeLa |
| Source |
|
Movie
Controller
Structure viewers
About Yorodumi Papers



Authors

External links




Homo sapiens (human)