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References from Regulation of cellular proteostasis via mitochondrial protein import. Local targets link to admitted publications; unresolved targets remain external evidence.
Mitochondria: in sickness and in health
10.1016/j.cell.2012.02.035 · 2012 · External reference
Mitochondrial protein import stress
10.1038/s41556-024-01590-w · 2025 · External reference
Interconnectivity of mitochondrial protein biogenesis and quality control
10.1016/j.tibs.2025.09.004 · 2025 · External reference
Mitochondrial machineries for protein import and assembly
10.1146/annurev-biochem-060815-014352 · 2017 · External reference
Quantitative high-confidence human mitochondrial proteome and its dynamics in cellular context
10.1016/j.cmet.2021.11.001 · 2021 · External reference
Definition of a high-confidence mitochondrial proteome at quantitative scale
10.1016/j.celrep.2017.06.014 · 2017 · External reference
A cytosolic network suppressing mitochondria-mediated proteostatic stress and cell death
10.1038/nature14859 · 2015 · External reference
Mistargeted mitochondrial proteins activate a proteostatic response in the cytosol
10.1038/nature14951 · 2015 · External reference
Mitochondrial protein import clogging as a mechanism of disease
10.7554/elife.84330 · 2023 · External reference
Cytosolic adaptation to mitochondria-induced proteostatic stress causes progressive muscle wasting
10.1016/j.isci.2021.103715 · 2022 · External reference
The mitochondrial import complex MIM functions as main translocase for α-helical outer membrane proteins
10.1016/j.celrep.2020.107567 · 2020 · External reference
Mitochondrial sorting and assembly machinery operates by β-barrel switching
10.1038/s41586-020-03113-7 · 2021 · External reference
Protein insertion into the mitochondrial inner membrane by a twin-pore translocase
10.1126/science.1080945 · 2003 · External reference
Mia40 targets cysteines in a hydrophobic environment to direct oxidative protein folding in the mitochondria
10.1038/ncomms4041 · 2014 · External reference
Global analysis of the mitochondrial N-proteome identifies a processing peptidase critical for protein stability
10.1016/j.cell.2009.07.045 · 2009 · External reference
Widespread use of unconventional targeting signals in mitochondrial ribosome proteins
10.15252/embj.2021109519 · 2022 · External reference
Structural basis of mitochondrial protein import by the TIM23 complex
10.1038/s41586-023-06239-6 · 2023 · External reference
Central role of Tim17 in mitochondrial presequence protein translocation
10.1038/s41586-023-06477-8 · 2023 · External reference
The MIA pathway: a key regulator of mitochondrial oxidative protein folding and biogenesis
10.1021/acs.accounts.5b00150 · 2015 · External reference
Targeting and insertion of membrane proteins in mitochondria
10.3389/fcell.2021.803205 · 2021 · External reference
Cryo-EM structure of the mitochondrial protein-import channel TOM complex at near-atomic resolution
10.1038/s41594-019-0339-2 · 2019 · External reference
Structure of the mitochondrial import gate reveals distinct preprotein paths
10.1038/s41586-019-1680-7 · 2019 · External reference
Cryo-EM structure of the TOM core complex from Neurospora crassa
10.1016/j.cell.2017.07.012 · 2017 · External reference
Tom5 functionally links mitochondrial preprotein receptors to the general import pore
10.1038/40663 · 1997 · External reference
Tom7 modulates the dynamics of the mitochondrial outer membrane translocase and plays a pathway-related role in protein import
10.1002/j.1460-2075.1996.tb00566.x · 1996 · External reference
Structural basis of presequence recognition by the mitochondrial protein import receptor Tom20
10.1016/s0092-8674(00)80691-1 · 2000 · External reference
The intermembrane space domain of mitochondrial Tom22 functions as a trans binding site for preproteins with N-terminal targeting sequences
10.1128/mcb.17.11.6574 · 1997 · External reference
Dynamics of the TOM complex of mitochondria during binding and translocation of preproteins
10.1128/mcb.18.9.5256 · 1998 · External reference
The chaperone-binding activity of the mitochondrial surface receptor Tom70 protects the cytosol against mitoprotein-induced stress
10.1016/j.celrep.2021.108936 · 2021 · External reference
Roles of Tom70 in import of presequence-containing mitochondrial proteins
10.1074/jbc.m109.041756 · 2009 · External reference
Unraveling the intricate organization of mammalian mitochondrial presequence translocases: existence of multiple translocases for maintenance of mitochondrial function
10.1128/mcb.01527-13 · 2014 · External reference
Mitochondrial YME1L1 governs unoccupied protein translocase channels
10.1038/s41556-024-01571-z · 2025 · External reference
Stress-regulated translational attenuation adapts mitochondrial protein import through Tim17A degradation
10.1016/j.cmet.2013.11.006 · 2013 · External reference
Lipid signalling drives proteolytic rewiring of mitochondria by YME1L
10.1038/s41586-019-1738-6 · 2019 · External reference
HSF1 remodels mitochondrial biogenesis and function in cancer cells via TIMM17A
2025 · External reference
Tim50 is a subunit of the TIM23 complex that links protein translocation across the outer and inner mitochondrial membranes
10.1016/s0092-8674(02)01053-x · 2002 · External reference
Structural basis for the function of Tim50 in the mitochondrial presequence translocase
10.1016/j.jmb.2011.06.020 · 2011 · External reference
The mitochondrial presequence translocase: an essential role of Tim50 in directing preproteins to the import channel
10.1016/s0092-8674(02)01073-5 · 2002 · External reference
ROMO1 is a constituent of the human presequence translocase required for YME1L protease import
10.1083/jcb.201806093 · 2019 · External reference
Mgr2 functions as lateral gatekeeper for preprotein sorting in the mitochondrial inner membrane
10.1016/j.molcel.2014.10.010 · 2014 · External reference
Interaction of the J-protein heterodimer Pam18/Pam16 of the mitochondrial import motor with the translocon of the inner membrane
10.1091/mbc.e07-08-0748 · 2008 · External reference
The presequence translocase-associated protein import motor of mitochondria: Pam16 functions in an antagonist manner to Pam18
10.1074/jbc.m404319200 · 2004 · External reference
Pam16 has an essential role in the mitochondrial protein import motor
10.1038/nsmb735 · 2004 · External reference
A J-protein is an essential subunit of the presequence translocase-associated protein import motor of mitochondria
10.1083/jcb.200308004 · 2003 · External reference
Tim14, a novel key component of the import motor of the TIM23 protein translocase of mitochondria
10.1093/emboj/cdg485 · 2003 · External reference
J protein cochaperone of the mitochondrial inner membrane required for protein import into the mitochondrial matrix
10.1073/pnas.1936150100 · 2003 · External reference
Mitochondrial Hsp70/MIM44 complex facilitates protein import
10.1038/371768a0 · 1994 · External reference
The nucleotide exchange factor MGE exerts a key function in the ATP-dependent cycle of mt-Hsp70-Tim44 interaction driving mitochondrial protein import
10.1002/j.1460-2075.1996.tb00966.x · 1996 · External reference
Protein folding in mitochondria requires complex formation with hsp60 and ATP hydrolysis
10.1038/341125a0 · 1989 · External reference
Mitochondrial heat-shock protein hsp60 is essential for assembly of proteins imported into yeast mitochondria
10.1038/337620a0 · 1989 · External reference
The YTA10–12 complex, an AAA protease with chaperone-like activity in the inner membrane of mitochondria
10.1016/s0092-8674(00)81271-4 · 1996 · External reference
Variable and tissue-specific subunit composition of mitochondrial m-AAA protease complexes linked to hereditary spastic paraplegia
10.1128/mcb.01470-06 · 2007 · External reference
Structure of the mitochondrial inner membrane AAA+ protease YME1 gives insight into substrate processing
10.1126/science.aao0464 · 2017 · External reference
Requirement for the yeast gene LON in intramitochondrial proteolysis and maintenance of respiration
10.1126/science.8146662 · 1994 · External reference
Structures of the human LONP1 protease reveal regulatory steps involved in protease activation
10.1038/s41467-021-23495-0 · 2021 · External reference
LONP1 and mtHSP70 cooperate to promote mitochondrial protein folding
10.1038/s41467-020-20597-z · 2021 · External reference
Functional proteolytic complexes of the human mitochondrial ATP-dependent protease, hClpXP
10.1074/jbc.m201642200 · 2002 · External reference
Cryo-EM structure of the ClpXP protein degradation machinery
10.1038/s41594-019-0304-0 · 2019 · External reference
Mitochondrial ClpX activates an essential biosynthetic enzyme through partial unfolding
10.7554/elife.54387 · 2020 · External reference
The multifaceted mitochondrial OXA insertase
10.1016/j.tcb.2023.02.001 · 2023 · External reference
Ribosome-associated quality-control mechanisms from bacteria to humans
10.1016/j.molcel.2022.03.038 · 2022 · External reference
CAT-tailing as a fail-safe mechanism for efficient degradation of stalled nascent polypeptides
10.1126/science.aam7787 · 2017 · External reference
Convergence of mammalian RQC and C-end rule proteolytic pathways via alanine tailing
10.1016/j.molcel.2021.03.004 · 2021 · External reference
A ribosome-bound quality control complex triggers degradation of nascent peptides and signals translation stress
10.1016/j.cell.2012.10.044 · 2012 · External reference
Cytosolic protein Vms1 links ribosome quality control to mitochondrial and cellular homeostasis
10.1016/j.cell.2017.10.002 · 2017 · External reference
Vms1 and ANKZF1 peptidyl-tRNA hydrolases release nascent chains from stalled ribosomes
10.1038/s41586-018-0022-5 · 2018 · External reference
Mitochondrial complexome reveals quality-control pathways of protein import
10.1038/s41586-022-05641-w · 2023 · External reference
Delayed protein translocation protects mitochondria against toxic CAT-tailed proteins
10.1016/j.molcel.2025.09.030 · 2025 · External reference
Cdc48-associated complex bound to 60S particles is required for the clearance of aberrant translation products
10.1073/pnas.1221724110 · 2013 · External reference
Mitochondrial protein translocation-associated degradation
10.1038/s41586-019-1227-y · 2019 · External reference
A UBH-UBX module amplifies p97/VCP's unfolding power to facilitate protein extraction and degradation
10.1038/s41467-025-65166-4 · 2025 · External reference
Substrate processing by the Cdc48 ATPase complex is initiated by ubiquitin unfolding
10.1126/science.aax1033 · 2019 · External reference
MitoCPR-A surveillance pathway that protects mitochondria in response to protein import stress
10.1126/science.aan4146 · 2018 · External reference
ATAD1 prevents clogging of TOM and damage caused by un-imported mitochondrial proteins
10.1016/j.celrep.2024.114473 · 2024 · External reference
Mechanism of phospho-ubiquitin-induced PARKIN activation
10.1038/nature14879 · 2015 · External reference
Binding to serine 65-phosphorylated ubiquitin primes Parkin for optimal PINK1-dependent phosphorylation and activation
10.15252/embr.201540352 · 2015 · External reference
Mitochondrial membrane potential regulates PINK1 import and proteolytic destabilization by PARL
10.1083/jcb.201008084 · 2010 · External reference
The mitophagy pathway and its implications in human diseases
10.1038/s41392-023-01503-7 · 2023 · External reference
Reinitiation involving upstream ORFs regulates ATF4 mRNA translation in mammalian cells
10.1073/pnas.0400541101 · 2004 · External reference
Regulated translation initiation controls stress-induced gene expression in mammalian cells
10.1016/s1097-2765(00)00108-8 · 2000 · External reference
Phosphorylation of eIF2 facilitates ribosomal bypass of an inhibitory upstream ORF to enhance CHOP translation
10.1074/jbc.m110.216093 · 2011 · External reference
A pathway coordinated by DELE1 relays mitochondrial stress to the cytosol
10.1038/s41586-020-2076-4 · 2020 · External reference
Mitochondrial stress is relayed to the cytosol by an OMA1–DELE1–HRI pathway
10.1038/s41586-020-2078-2 · 2020 · External reference
Cytosolic retention of HtrA2 during mitochondrial protein import stress triggers the DELE1-HRI pathway
10.1038/s42003-024-06107-7 · 2024 · External reference
HRI protein kinase in cytoplasmic heme sensing and mitochondrial stress response: relevance to hematological and mitochondrial diseases
10.1016/j.jbc.2025.108494 · 2025 · External reference
Mitochondrial import efficiency of ATFS-1 regulates mitochondrial UPR activation
10.1126/science.1223560 · 2012 · External reference
A cytosolic surveillance mechanism activates the mitochondrial UPR
10.1038/s41586-023-06142-0 · 2023 · External reference
Tom20 and Tom22 share the common signal recognition pathway in mitochondrial protein import
10.1074/jbc.m708339200 · 2008 · External reference
Regulation of mitochondrial protein import by the nucleotide exchange factors GrpEL1 and GrpEL2 in human cells
10.1074/jbc.m117.788463 · 2017 · External reference
Molecular pathway of mitochondrial preprotein import through the TOM–TIM23 supercomplex
10.1038/s41594-023-01103-7 · 2023 · External reference
Newly imported proteins in mitochondria are particularly sensitive to aggregation
10.1111/apha.13985 · 2023 · External reference
Mechanism of membrane-tethered mitochondrial protein synthesis
10.1126/science.abe0763 · 2021 · External reference
Molecular connectivity of mitochondrial gene expression and OXPHOS biogenesis
10.1016/j.molcel.2020.07.024 · 2020 · External reference
Early fate decision for mitochondrially encoded proteins by a molecular triage
10.1016/j.molcel.2023.09.001 · 2023 · External reference
Prohibitins regulate membrane protein degradation by the m-AAA protease in mitochondria
10.1128/mcb.19.5.3435 · 1999 · External reference
In situ architecture of the human prohibitin complex
10.1038/s41556-025-01620-1 · 2025 · External reference
Cryo-EM structure of the prohibitin complex in open conformation
10.1073/pnas.2512430122 · 2025 · External reference
Structural basis for prohibitin-mediated regulation of mitochondrial m-AAA protease
2025 · External reference
UPRmt scales mitochondrial network expansion with protein synthesis via mitochondrial import in Caenorhabditis elegans
10.1038/s41467-020-20784-y · 2021 · External reference
UPRmt regulation and output: a stress response mediated by mitochondrial-nuclear communication
10.1038/cr.2018.16 · 2018 · External reference
Dual functions of Mss51 couple synthesis of Cox1 to assembly of cytochrome c oxidase in Saccharomyces cerevisiae mitochondria
10.1091/mbc.e09-06-0522 · 2009 · External reference
The Cbp3–Cbp6 complex coordinates cytochrome b synthesis with bc1 complex assembly in yeast mitochondria
10.1083/jcb.201206040 · 2012 · External reference
The translational activator Sov1 coordinates mitochondrial gene expression with mitoribosome biogenesis
10.1093/nar/gkaa424 · 2020 · External reference
Assembly-dependent translation of subunits 6 (Atp6) and 9 (Atp9) of ATP synthase in yeast mitochondria
10.1093/genetics/iyac007 · 2022 · External reference
Molecular wiring of a mitochondrial translational feedback loop
10.1016/j.molcel.2019.11.019 · 2020 · External reference
A molecular switch at the yeast mitoribosomal tunnel exit controls cytochrome b synthesis
10.1093/nar/gkaf634 · 2025 · External reference
Synchronized mitochondrial and cytosolic translation programs
10.1038/nature18015 · 2016 · External reference
Mitochondrial protein synthesis adapts to influx of nuclear-encoded protein
10.1016/j.cell.2016.09.003 · 2016 · External reference
Structure prediction analysis of human core TIM23 complex reveals conservation of the protein translocation mechanism
10.1002/2211-5463.13840 · 2024 · External reference
OCIAD1 and prohibitins regulate the stability of the TIM23 protein translocase
10.1016/j.celrep.2024.115038 · 2024 · External reference
Stress adaptation of mitochondrial protein import by OMA1-mediated degradation of DNAJC15
10.1038/s41594-026-01756-0 · 2026 · External reference
Redox regulation of GRPEL2 nucleotide exchange factor for mitochondrial HSP70 chaperone
10.1016/j.redox.2018.07.024 · 2018 · External reference
Preferential binding of ADP-bound mitochondrial HSP70 to the nucleotide exchange factor GRPEL1 over GRPEL2
10.1002/pro.5190 · 2024 · External reference
TIMM17A overexpression in lung adenocarcinoma and its association with prognosis
10.1038/s41598-024-59526-1 · 2024 · External reference
Functional diversity of human mitochondrial J-proteins is independent of their association with the inner membrane presequence translocase
10.1074/jbc.m116.738146 · 2016 · External reference
Cleaning up: ER-associated degradation to the rescue
10.1016/j.cell.2012.11.012 · 2012 · External reference
Dynamic regulation of mitochondrial import by the ubiquitin system
10.1016/j.molcel.2020.02.012 · 2020 · External reference
Ubx2 links the Cdc48 complex to ER-associated protein degradation
10.1038/ncb1298 · 2005 · External reference
Membrane-bound Ubx2 recruits Cdc48 to ubiquitin ligases and their substrates to ensure efficient ER-associated protein degradation
10.1038/ncb1299 · 2005 · External reference
A direct role for a mitochondrial targeting sequence in signalling stress
10.1038/s41586-025-09834-x · 2026 · External reference
Targeting and plasticity of mitochondrial proteins revealed by proximity-specific ribosome profiling
10.1126/science.1257522 · 2014 · External reference
Mechanisms and functions of ribosome-associated protein quality control
10.1038/s41580-019-0118-2 · 2019 · External reference
Failure of RQC machinery causes protein aggregation and proteotoxic stress
10.1038/nature16973 · 2016 · External reference
Release of ubiquitinated and non-ubiquitinated nascent chains from stalled mammalian ribosomal complexes by ANKZF1 and Ptrh1
10.1016/j.molcel.2018.08.022 · 2018 · External reference
NEMF-mediated listerin-independent mitochondrial translational surveillance by E3 ligase Pirh2 and mitochondrial protease ClpXP
10.1016/j.celrep.2024.113860 · 2024 · External reference
Compromised mitochondrial protein import acts as a signal for UPRmt
10.1016/j.celrep.2019.07.049 · 2019 · External reference
ClpP mediates activation of a mitochondrial unfolded protein response in C. elegans
10.1016/j.devcel.2007.07.016 · 2007 · External reference
Selective induction of mitochondrial chaperones in response to loss of the mitochondrial genome
10.1111/j.1432-1033.1996.0098h.x · 1996 · External reference
Compartment-specific perturbation of protein handling activates genes encoding mitochondrial chaperones
10.1242/jcs.01275 · 2004 · External reference
Mitonuclear protein imbalance as a conserved longevity mechanism
10.1038/nature12188 · 2013 · External reference
Mitochondrial unfolded protein response controls matrix pre-RNA processing and translation
10.1038/nature18302 · 2016 · External reference
A mitochondrial specific stress response in mammalian cells
10.1093/emboj/cdf445 · 2002 · External reference
Mitochondrial and nuclear accumulation of the transcription factor ATFS-1 promotes OXPHOS recovery during the UPRmt
10.1016/j.molcel.2015.02.008 · 2015 · External reference
The UPRmt preserves mitochondrial import to extend lifespan
10.1083/jcb.202201071 · 2022 · External reference
Mitochondrial proteostasis in the context of cellular and organismal health and aging
10.1074/jbc.tm117.000893 · 2019 · External reference
Folding the mitochondrial UPR into the integrated stress response
10.1016/j.tcb.2020.03.001 · 2020 · External reference
The transcription factor ATF5 mediates a mammalian mitochondrial UPR
10.1016/j.cub.2016.06.002 · 2016 · External reference
HSP70 protein promotes survival of C6 and U87 glioma cells by inhibition of ATF5 degradation
10.1074/jbc.m110.211771 · 2011 · External reference
Phosphorylation of eIF2 directs ATF5 translational control in response to diverse stress conditions
10.1074/jbc.m708530200 · 2008 · External reference
HSF1 at a glance
10.1242/jcs.132605 · 2014 · External reference
A mitochondrial iron-responsive pathway regulated by DELE1
10.1016/j.molcel.2023.05.031 · 2023 · External reference
Adaptation to mitochondrial stress requires CHOP-directed tuning of ISR
10.1126/sciadv.abf0971 · 2021 · External reference
PINK1 is selectively stabilized on impaired mitochondria to activate Parkin
10.1371/journal.pbio.1000298 · 2010 · External reference
Tom20 gates PINK1 activity and mediates its tethering of the TOM and TIM23 translocases upon mitochondrial stress
10.1073/pnas.2313540121 · 2024 · External reference
High-content genome-wide RNAi screens identify regulators of parkin upstream of mitophagy
10.1038/nature12748 · 2013 · External reference
TIM23 facilitates PINK1 activation by safeguarding against OMA1-mediated degradation in damaged mitochondria
10.1016/j.celrep.2023.112454 · 2023 · External reference
Reciprocal roles of Tom7 and OMA1 during mitochondrial import and activation of PINK1
10.1016/j.molcel.2019.01.002 · 2019 · External reference
PINK1 autophosphorylation upon membrane potential dissipation is essential for Parkin recruitment to damaged mitochondria
10.1038/ncomms2016 · 2012 · External reference
PINK1 is activated by mitochondrial membrane potential depolarization and stimulates Parkin E3 ligase activity by phosphorylating serine 65
10.1098/rsob.120080 · 2012 · External reference
A Ubl/ubiquitin switch in the activation of Parkin
10.15252/embj.201592237 · 2015 · External reference
Protein import motor complex reacts to mitochondrial misfolding by reducing protein import and activating mitophagy
10.1038/s41467-022-32564-x · 2022 · External reference
E2F coregulates an essential HSF developmental program that is distinct from the heat-shock response
10.1101/gad.283317.116 · 2016 · External reference
Mitochondrial stress induces chromatin reorganization to promote longevity and UPRmt
10.1016/j.cell.2016.04.011 · 2016 · External reference
Mitochondrial stress restores the heat shock response and prevents proteostasis collapse during aging
10.1016/j.celrep.2017.10.038 · 2017 · External reference
Neuroendocrine coordination of mitochondrial stress signaling and proteostasis
10.1016/j.cell.2016.08.042 · 2016 · External reference
Neuropeptide signals cell non-autonomous mitochondrial unfolded protein response
10.1038/cr.2016.118 · 2016 · External reference
The mitochondrial unfolded protein response is mediated cell-non-autonomously by retromer-dependent Wnt signaling
10.1016/j.cell.2018.06.029 · 2018 · External reference
Autophagy deficiency leads to protection from obesity and insulin resistance by inducing Fgf21 as a mitokine
10.1038/nm.3014 · 2013 · External reference
