Research Summary

Mitochondria are organelles that serve as a hub for cellular signaling and the biogenesis of indispensable metabolites. We aim to understand how mitochondrial homeostasis is maintained under challenging physiological and disease conditions.

Even though mitochondria contain their own genome, 99% of their proteome is encoded by the eukaryotic genome. Therefore, the constant import of proteins into the organelle is essential to eukaryotic cell function. Perturbations in this process cause multiple human disorders and are associated with aging and neurodegenerative diseases.

We discovered a cellular response to protein import defects which we have termed the mitoCPR (mitochondrial compromised protein import response). The mitoCPR maintains mitochondrial functionality during import stress and prevents permanent damage to the mitochondrial DNA.

Our lab employs cell biology, biochemical and genetic approaches to investigate how cells cope with defects in protein import. Using budding yeast and mammalian cells we aim to elucidate the molecular mechanisms that ensure mitochondrial health in the face of import defects.

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Mitochondria to nucleus signaling: Communication between mitochondria and the nucleus is crucial for maintaining mitochondrial function and, consequently, for cell viability. We study the signaling route between these organelles during protein import stress. Our goal is to identify the mitochondrial signal that is sensed by cells to activate the mitoCPR.

Mitochondrial protein quality control: During import stress un-imported proteins stall in the mitochondrial import channels. One function of the mitoCPR is to alleviate this stress by extracting stalled proteins from the channels. We aim to elucidate the mechanisms by which proteins are recognized and extracted from the membrane during import stress.

Mitochondrial import stress in mammals: Impaired mitochondrial protein import is a hallmark of several human disorders and neurodegenerative diseases. We are interested in understanding how mammalian cells cope with protein import defects. 



Postdoctoral fellow in the Amon laboratory at The Massachusetts Institute of Technology

PhD in the Elazar laboratory at The Weizmann Institute of Science, Israel

MSc in the Horowitz laboratory at Tel Aviv University, Israel


The Jane Coffin Childs Memorial Fund for Medical Research Fellowship (2013)

EMBO Long-Term Fellowship (2012)

Israeli National Postdoctoral Award for Advancing Women in Science (2011)      

Lady Anne Chain Memorial Prize for PhD students (2011)

  1. Weidberg H, Amon, A. (2018) MitoCPR—A surveillance pathway that protects mitochondria in response to protein import stress. Science eaan4146 360.
  2. Rapaport D, Fichtman B, Weidberg H, Sprecher E, Horowitz M. (2018) NEK3-mediated SNAP29 phosphorylation modulates its membrane association and SNARE fusion dependent processes. Biochem Biophys Res Commun. 497(2):605-611
  3. Weidberg H, Moretto F, Spedale G, Amon A,  J. van Werven F. (2016) Nutrient control of yeast gametogenesis is mediated by TORC1, PKA and energy availability. PLoS Genet. 12(6).
  4. Pekar O, Benjamin S, Weidberg H, Smaldone S, Ramirez F, Horowitz M. (2012) EHD2 shuttles to the nucleus and represses transcription. Biochem J. 444, 383-94.
  5. Weidberg H, Shpilka T, Shvets E, Abada Adi, Shimron F, Elazar Z. (2011) LC3 and GATE-16 N-termini mediate membrane fusion processes required for autophagosome biogenesis. Dev Cell, 20, 444-54.
  6. Weidberg H, Shvets E, Elazar, Z. (2011) Biogenesis and cargo selectivity of autophagosomes. Ann Rev Biochem, 80125-56.
  7. Shvets E, Abada A, Weidberg H, Elazar Z. (2011) Dissecting the involvement of LC3B and GATE-16 in p62 recruitment into autophagosomes. Autophagy, 7, 683-8.
  8. Weidberg H, Elazar Z. (2011) TBK1 mediates cross-talk between the innate immune response and autophagy. Sci Signal, 4.
  9.  Shpilka T, Weidberg H, Pietrokovski S, Elazar Z. (2011) Atg8, an autophagic-related ubiquitin-like protein family. Genome Biol, 12, 226. 
  10. Benjamin S, Weidberg H, Rapaport D, Pekar O, Nudelman M, Segal D, Hirschberg K, Katzav S, Ehrlich M, Horowitz M. (2011) EHD2 mediates trafficking from the plasma membrane by modulating Rac1 activity. Biochem J., 439, 433-42.
  11. Weidberg H, Shpilka T, Shvets E, Elazar Z. (2010) Mammalian Atg8s: one is simply not enough. Autophagy 6, 808-9.
  12. Weidberg H, Shvets E, Shpilka T, Shimron F, Shinder V, Elazar Z. (2010) LC3 and GATE-16/GABARAP subfamilies are both essential yet act differently in autophagosome biogenesis. Embo J, 29, 1792-802.
  13. Scherz-Shouval R, Weidberg H, Gonen C, Wilder S, Elazar Z, Oren M. (2010) p53-dependent regulation of autophagy provides cancer cells with a survival advantage under prolonged starvation. PNAS, 43, 18511-6. 
  14. Weidberg H, Shvets E, Elazar Z. (2009) Lipophagy: selective catabolism designed for lipids. Dev Cell, 16, 628-30.
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