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This paper examines the political attributes of emigrants and how their departure affects the electoral outcomes in their home countries. I argue that emigrants are different from those who remain in their political preferences as well as economic attributes, such that large-scale emigration changes the distribution of voters in sending countries. Emigration can also directly affect the policy preferences of individuals who stay in their home countries. I test these arguments in seven Central and Eastern European countries, using individual-level surveys and region-level data on emigration and elections. To address potential endogeneity issues, I use instrumental variable analysis, leveraging the surge of Polish emigration to the United Kingdom after the EU enlargement. I find that emigrants from Central and Eastern Europe tend to be younger, highly educated, and politically more progressive and that the vote shares of far-right parties are larger in regions with higher emigration rates. Also, I find that exposure to large-scale emigration affects the vote choices of individuals who remain.

B cell development is linked to successful V(D)J recombination, allowing B cell receptor expression and ultimately antibody secretion for adaptive immunity. Germline noncoding RNAs (ncRNAs) are produced at immunoglobulin (Ig) loci during V(D)J recombination, but their function and posttranscriptional regulation are incompletely understood. Patients with trichohepatoenteric syndrome, characterized by RNA exosome pathway component mutations, exhibit lymphopenia, thus demonstrating the importance of ncRNA surveillance in B cell development in humans. To understand the role of RNA exosome in early B cell development in greater detail, we generated mouse models harboring a B cell–specific cre allele ( Mb1 cre ), coupled to conditional inversion-deletion alleles of one RNA exosome core component ( Exosc3 ) or RNase catalytic subunits ( Exosc10 or Dis3 ). We noticed increased expression of RNA exosome subunits during V(D)J recombination, whereas a B cell developmental blockade at the pro–B cell stage was observed in the different knockout mice, overlapping with a lack of productive rearrangements of VDJ genes at the Ig heavy chain ( Igh ). This unsuccessful recombination prevented differentiation into pre–B cells, with accumulation of ncRNAs and up-regulation of the p53 pathway. Introduction of a prearranged Igh VDJ allele partly rescued the pre–B cell population in Dis3 -deficient cells, although V-J recombination defects were observed at Ig light chain kappa ( Ig κ), preventing subsequent B cell development. These observations demonstrated that the RNA exosome complex is important for Igh and Ig κ recombination and establish the relevance of RNA processing for optimal diversification at these loci during B cell development.

Atmospheric plasmas have been applied for the inactivation of microorganisms. Industrials demand to investigate the relation of the key reactive species induced by plasmas and the operating parameters including boundary conditions in order to control plasma treatment processes. In this study, we investigated the effect of gap distance between a pin-electrode and water surface on inactivation efficacy. When the gap distance decreased from 5 mm to 1 mm, the reduction of

A sizeable literature has emerged over the past several decades which examines the impact of adult civic education on democratic citizenship in emerging democracies. In this paper we describe the goals, procedures and the results of a systematic review and meta-analysis of the overall effects of civic education interventions on outcomes relevant for support for democratic political systems, norms and values. We extract information from 25 articles that report results from 30 different experimental interventions. The analyses focus on the effectiveness of these interventions in non-established democracies, where democratic political culture is typically more vulnerable and hence democracy promotion most important. We discuss the primary features of civic education studies in terms of their geographic location, their sample populations, the types of interventions employed and outcomes that are considered. Our meta-analysis demonstrates an unusually high heterogeneous treatment effects, with some studies leading to strong negative impacts while others strongly boost democratic support.

Can democratic orientations and political participation in fragile democracies be fostered through civic education? Early evaluation work reported generally positive effects, though recent work has been more skeptical, with some studies reporting negative impacts of civic education on political engagement through highlighting the poor performance of incumbents and ongoing political processes. In this article, we report the results of a field experiment using an encouragement design to assess the Voter Opinion and Involvement through Civic Education (VOICE) programme conducted in 2010-2011 in the Democratic Republic of Congo. We adapt Bratton and Mattes’ (2007) “supply and demand” model of democratic support to the case of civic education, and derive hypotheses regarding expected impacts of VOICE on a series of democratic orientations and political participation. The results show that the VOICE programme had negative effects on support for the decentralization process and on individuals’ satisfaction with democracy in the DRC, and positive effects on non-electoral participation as well as on democratic orientations such as knowledge, efficacy, and political tolerance. We suggest that this pattern of effects has positive normative implications, and that civic education programmes continue to have the potential to deepen democratic engagement and values, even in fragile or backsliding democratic settings.

Tobacco is an excellent model plant to study developmental processes and cellular biology. Surface dielectric barrier discharge (sDBD) was used to generate plasma activated water (PAW), and the PAW was indirectly applied to study tobacco plant growth during the early developmental stages. Solid media were treated with the PAW for 8- and 18-day-old seedlings, and the effects on both the vegetative and root phenotypes were observed during the early developmental stages. In the vegetative tissue, the cotyledon size was large for the seedling that was cultivated by plasma activated water for the 12 min PAW (PAW12) treatment. The PAW dramatically elongated the cotyledon by 40% due to increased cell expansion in the leaf-length direction, not cell proliferation. In addition to the vegetative tissue, the root length due to the PAW was significantly enhanced at the primary root with elongated root hairs. According to molecular analysis, the NT_ COBRA-LIKE 9 (COBL) and two NT_ XYLOGLUCAN ENDO-TRANSGLYCOSYLASE/HYDROLASE (XTH) genes, NT_XTH9 and NT_XTH15, were significantly over-expressed by the PAW treatment compared with untreated deionized water (DW), indicating that the PAW effects are from modulating the expression of target genes. These results demonstrate that PAW might promote tobacco plant growth both in the cotyledon and root hair tissues via the NT_COBL, NT_XTH9 and NT_XTH15 genes.

International audience; During transcription and replication, R-loops that contain RNA-DNA hybrids are generated across numerous genomic loci and contribute to many biological events. Using S9.6, a monoclonal antibody against RNA-DNA hybrids, accelerated the study of R-loop biology. An outpouring of recent studies has implicated various contributions of R-loop in physiological cellular functions. Earlier studies using nondenaturing sodium bisulfite probing also supported existence of DNA-RNA hybrids formation in mammalian cells. In activated B cells, RNA-DNA hybrids formation at IgH gene locus of B cells is crucial for class switch recombination that ensure the proper effector function of the antibody. Here, we describe the identification of R-loops associated with the IgH locus using RNA-DNA hybrid immunoprecipitation sequencing and nondenaturing sodium bisulfite probing. This will be helpful for future studies of R-loops status on whole genome as well as on IgH locus in B cells.

VPS34 is a class III phosphoinositide 3-kinase involved in endosomal trafficking and autophagosome formation. Inhibitors of VPS34 were believed to have value as anticancer agents, but genetic and pharmacological data suggest that sustained inhibition of VPS34 kinase activity may not be well tolerated. Here we disclose the identification of a novel series of dihydropyrazolopyrazinone compounds represented by compound 5 as potent, selective, and orally bioavailable VPS34 inhibitors through a structure-based design strategy. A water-interacting hydrogen bond acceptor within an appropriate distance to a hinge-binding element was found to afford significant VPS34 potency across chemical scaffolds. The selectivity of compound 5 over PIK family kinases arises from interactions between the hinge-binding element and the pseudo-gatekeeper residue Met682. As recent in vivo pharmacology data suggests that sustained inhibition of VPS34 kinase activity may not be tolerated, structure-activity relationships leading to VPS34 inhibition may be helpful for avoiding this target in other ATP-competitive kinase programs.

BACKGROUND & AIMS: Adiposity has been consistently associated with gallstone disease risk. We aimed to characterize associations of anthropometric measures (body mass index [BMI], recent weight change, long-term weight change, waist circumference, and waist-to-hip ratio) with symptomatic gallstone disease according to strata of gallstone disease polygenic risk score (PRS). METHODS: We conducted analysis among 34,626 participants with available genome-wide genetic data within three large, prospective, US cohorts – Nurses’ Health Study (NHS), Health Professionals Follow-up Study, and NHS II. We characterized joint associations of PRS and anthropometric measures and tested for interactions on the relative and absolute risk scales. RESULTS: Women in the highest BMI and PRS categories (BMI ≥ 30 kg/m(2) and PRS ≥ 1 SD above mean) had odds ratio (OR) for gallstone disease of 5.55 (95% CI, 5.29–5.81) compared with those in the lowest BMI and PRS categories (BMI < 25 kg/m(2) and PRS < 1 SD below mean). The corresponding OR among men was 1.65 (95% CI, 1.02–2.29). Associations for BMI did not vary within strata of PRS on the relative risk scale. On the absolute risk scale, the incidence rate difference between obese and normal-weight individuals was 1086/100,000 PYs within the highest PRS category, compared to 666/100,000 PYs in the lowest PRS category, with strong evidence for interaction with the ABCG8 locus. CONCLUSIONS: While maintenance of a healthy body weight reduces gallstone disease risk among all individuals, risk reduction is higher among the subset with greater genetic susceptibility to gallstone disease.

Improving the microbiological quality of salted Chinese cabbage, used in kimchi, an important part of the Asian culinary scene, is critical for minimising exposure to foodborne pathogens. This study evaluated the effects of a sequential combination of washing treatments using plasma-activated water (PAW), tap water, and sodium hypochlorite (NaOCl) and mild heating (MH) at 60 °C on the inactivation of background microbiota and inoculated foodborne pathogens and the quality of shredded salted Chinese cabbages. Treatment with 120-PAW alone resulted in 2.0, 2.2, 1.8, and 0.9 log CFU/g reduction in mesophilic aerobic bacteria, lactic acid bacteria, yeast and moulds, and coliforms, respectively, while subsequent MH treatment reduced the counts of lactic acid bacteria, yeast and moulds, and coliforms to below detection limits. Sequential combination of washing with 120-PAW followed by MH caused 3.4 and 3.7 log CFU/g reductions in Listeria monocytogenes and Staphylococcus aureus inoculated into salted Chinese cabbages. The combined treatment caused negligible changes in moisture content, reducing sugar content, instrumental hardness, and CIE colour values. The salinity and peroxidase activity were significantly (P

An effective and eco-friendly technology is needed to prevent postharvest loss of onion bulbs during cold storage. This study investigated the effect of gaseous ozone on the decay and quality of onion bulbs during storage at 2 °C and 70% relative humidity for two months. Gaseous ozone was adjusted to a concentration of 1.27 ± 0.024 ppm in the storage room by generating a high voltage discharge in air. After two months of storage, gaseous ozone significantly reduced the counts of aerobic bacteria (e.g., Rahnella aquatilis) and fungi (e.g., yeast and mold) in the onion bulbs by 4 log (CFU g−1) and 0.92 log (CFU g−1) compared with those of an untreated control, respectively. The microbial reduction by gaseous ozone resulted in a lower rotten rate of the onion bulbs, which was less than 20.0% compared with that of the untreated control. Moreover, the ozone exposure extended the storage life of the onion bulbs by delaying its color change and softening during storage. Our results suggest that gaseous ozone can control the decay of onion bulbs safely during storage.

Noncoding RNAs are exquisitely titrated by the cellular RNA surveillance machinery for regulating diverse biological processes. The RNA exosome, the predominant 3' RNA exoribonuclease in mammalian cells, is composed of nine core and two catalytic subunits. Here, we developed a mouse model with a conditional allele to study the RNA exosome catalytic subunit DIS3. In DIS3-deficient B cells, integrity of the immunoglobulin heavy chain (Igh) locus in its topologically associating domain is affected, with accumulation of DNA-associated RNAs flanking CTCF-binding elements, decreased CTCF binding to CTCF-binding elements and disorganized cohesin localization. DIS3-deficient B cells also accumulate activation-induced cytidine deaminase-mediated asymmetric nicks, altering somatic hypermutation patterns and increasing microhomology-mediated end-joining DNA repair. Altered mutation patterns and Igh architectural defects in DIS3-deficient B cells lead to decreased class-switch recombination but increased chromosomal translocations. Our observations of DIS3-mediated architectural regulation at the Igh locus are reflected genome wide, thus providing evidence that noncoding RNA processing is an important mechanism for controlling genome organization.

Gamma-aminobutyric acid (GABA) is a natural amino acid with antioxidant activity and is often considered to have therapeutic potential against obesity. Obesity has long been linked to ROS and ER stress, but the effect of GABA on the ROS-associated ER stress axis has not been thoroughly explored. Thus, in this study, the effect of GABA and fermented Curcuma longa L. extract enriched with GABA (FCLL-GABA) on the ROS-related ER stress axis and inositol-requiring transmembrane kinase/endoribonuclease 1α (IRE1α) sulfonation were examined with the HFD model to determine the underlying anti-obesity mechanism. Here, GABA and FCLL-GABA supplementations significantly inhibited the weight gain in HFD fed mice. The GABA and FCLL-GABA supplementation lowered the expressions of adipogenic transcription factors such as PPAR-γ, C/EBPα, FAS, and SREBP-1c in white adipose tissue (WAT) and liver from HFD-fed mice. The enhanced hyper-nutrient dysmetabolism-based NADPH oxidase (Nox) 4 and the resultant IRE1α sulfonation-RIDD-SIRT1 decay under HFD conditions were controlled with GABA and FCLL-GABA. Notably, GABA and FCLL-GABA administration significantly increased AMPK and sirtuin 1 (SIRT1) levels in WAT of HFD-fed mice. These significant observations indicate that ER-localized Nox4-induced IRE1α sulfonation results in the decay of SIRT1 as a novel mechanism behind the positive implications of GABA on obesity. Moreover, the investigation lays a firm foundation for the development of FCLL-GABA as a functional ingredient.

Defective autophagy is associated with chronic inflammation. Loss-of-function of the core autophagy gene Atg16l1 increases risk for Crohn’s disease by enhancing innate immunity in macrophages. However, autophagy also mediates clearance of intracellular pathogens. These divergent observations prompted a re-evaluation of ATG16L1 in antimicrobial immunity. In this study, we found that loss of Atg16l1 in macrophages enhanced the killing of virulent Shigella flexneri (S.flexneri), an enteric bacterium that resides within the cytosol by escaping all membrane-bound compartments. Quantitative multiplexed proteomics revealed that ATG16L1 deficiency significantly upregulated proteins involved in the glutathione-mediated antioxidant response to compensate for elevated oxidative stress, which also promoted S.flexneri killing. Consistently, myeloid cell-specific deletion of Atg16l1 accelerated bacterial clearance in vivo. Finally, pharmacological modulation of oxidative stress by suppression of cysteine import conferred enhanced microbicidal properties to wild type macrophages. These findings demonstrate that control of oxidative stress by ATG16L1 regulates antimicrobial immunity against intracellular pathogens.Impact statementMaculins et al utilize multiplexed mass spectrometry to show that loss of the autophagy gene Atg16l1 in macrophages enhances antimicrobial immunity against intracellular pathogens via the oxidative stress response.

To reduce hydrological disasters, it is necessary to operate rain gauge stations at locations where the spatio-temporal characteristics of rainfall can be reflected. Entropy has been widely used to evaluate the designs and uncertainties associated with rain gauge networks. In this study, the optimal rain gauge network in the Daegu and Gyeongbuk area, which requires the efficient use of water resources due to low annual precipitation and severe drought damage, was determined using conditional and joint entropy, and the selected network was quantitatively evaluated using the root mean square error (RMSE). To consider spatial distribution, prediction errors were generated using kriging. Four estimators used in entropy calculations were compared, and weighted entropy was calculated by weighting the precipitation. The optimal number of rain gauge stations was determined by calculating the RMSE reduction and the reduction ratio according to the number of selected rain gauge stations. Our findings show that the results of conditional entropy were better than those of joint entropy. The optimal rain gauge stations showed a tendency wherein peripheral rain gauge stations were selected first, with central stations being added afterward.

Loss of function of senataxin (SETX), a bona-fide RNA/DNA helicase, is associated with neuronal degeneration leading to Ataxia and Ocular Apraxia (AOA) in human patients. SETX is proposed to promote transcription termination, DNA replication, DNA repair, and to unwind deleterious RNA:DNA hybrids in the genome. In all the above-mentioned mechanisms, SETX unwinds transcription complex-associated nascent RNA which is then degraded by the RNA exosome complex. Here we have used B cells isolated from a SETX mutant mouse model and compared genomic instability and immunoglobulin heavy chain locus (IgH) class switch recombination (CSR) to evaluate aberrant and programmed genomic rearrangements, respectively. Similar to RNA exosome mutant primary B cells, SETX mutant primary B cells display genomic instability but a modest decrease in efficiency of CSR. Furthermore, knockdown of Setx mRNAs from CH12–F3 B-cell lines leads to a defect in IgA CSR and accumulation of aberrant patterns of mutations in IgH switch sequences. Given that SETX mutant mice do not recapitulate the AOA neurodegenerative phenotype, it is possible that some aspects of SETX biology are rescued by redundant helicases in mice. Overall, our study provides new insights into the role of the SETX/RNA exosome axis in suppressing genomic instability so that programmed DNA breaks are properly orchestrated., Biological sciences; Immunology; Genetics; Biochemistry; Molecular biology; RNA exosome, Senataxin, DNA/RNA hybrids, Class switch recombination

B cells undergo two types of genomic alterations to increase antibody diversity: introduction of point mutations into immunoglobulin heavy- and light-chain (IgH and IgL) variable regions by somatic hypermutation (SHM) and alteration of antibody effector functions by changing the expressed IgH constant region exons through IgH class switch recombination (CSR). SHM and CSR require the B cell–specific activation-induced cytidine deaminase (AID) protein, the transcription of germline noncoding RNAs, and the activity of the 3′ regulatory region (3′RR) super-enhancer. Although many transcription regulatory elements (e.g., promoters and enhancers) reside inside the IgH and IgL sequences, the question remains whether clusters of regulatory elements outside IgH control CSR. Using RNA exosome-deficient mouse B cells where long noncoding RNAs (lncRNAs) are easily detected, we identified a cluster of three RNA-expressing elements that includes lncCSR (IgA) (that expresses lncRNA-CSR(IgA)). B cells isolated from a mouse model lacking lncRNA-CSR(IgA) transcription fail to undergo normal levels of CSR to IgA both in B cells of the Peyer’s patches and grown in ex vivo culture conditions. lncRNA-CSR(IgA) is expressed from an enhancer site (lncCSR(IgA)) to facilitate the recruitment of regulatory proteins to a nearby CTCF site (CTCF(lncCSR)) that alters the chromosomal interactions inside the TAD(lncCSRIgA) and long-range interactions with the 3′RR super-enhancer. Humans with IgA deficiency show polymorphisms in the lncCSR(IgA) locus compared with the normal population. Thus, we provide evidence for an evolutionarily conserved topologically associated domain (TAD(lncCSRIgA)) that coordinates IgA CSR in Peyer’s patch B cells through an lncRNA (lncRNA-CSR(IgA)) transcription-dependent mechanism.

Immunoglobulin heavy chain (IgH) locus-associated G-rich long noncoding RNA (SμGLT) is important for physiological and pathological B cell DNA recombination. We demonstrate that the METTL3 enzyme-catalyzed N6-methyladenosine (m6A) RNA modification drives recognition and 3' end processing of SμGLT by the RNA exosome, promoting class switch recombination (CSR) and suppressing chromosomal translocations. The recognition is driven by interaction of the MPP6 adaptor protein with nuclear m6A reader YTHDC1. MPP6 and YTHDC1 promote CSR by recruiting AID and the RNA exosome to actively transcribe SμGLT. Direct suppression of m6A modification of SμGLT or of m6A reader YTHDC1 reduces CSR. Moreover, METTL3, an essential gene for B cell development in the bone marrow and germinal center, suppresses IgH-associated aberrant DNA breaks and prevents genomic instability. Taken together, we propose coordinated and central roles for MPP6, m6A modification, and m6A reader proteins in controlling long noncoding RNA processing, DNA recombination, and development in B cells.

PURPOSE Most studies on immune tolerance of mesenchymal stem cells (MSCs) have been performed using MSCs derived from bone marrow, cord blood, or adipose tissue. MSCs also exist in the craniofacial area, specifically in teeth. The purpose of this study was to evaluate the mechanisms of immune tolerance of dental pulp-derived MSC (DP-MSC) in vitro and in vivo. MATERIALS AND METHODS We isolated DP-MSCs from human dental pulp and co-cultured them with CD4⁺ T-cells. To evaluate the role of cytokines, we blocked TGF-β and IL-10, separately and together, in co-cultured DP-MSCs and CD4⁺ T-cells. We analyzed CD25 and FoxP3 to identify regulatory T-cells (Tregs) by fluorescence-activated cell sorting (FACS) and real-time PCR. We performed alloskin grafts with and without DP-MSC injection in mice. We performed mixed lymphocyte reactions (MLRs) to check immune tolerance. RESULTS Co-culture of CD4⁺ T-cells with DP-MSCs increased the number of CD4⁺CD25⁺FoxP3⁺ Tregs (p

The mechanisms by which lymphocytes engineer their genomes, allowing the adaptive immune system to respond to an enormous spectrum of Ags, have been one of the most exciting topics in immunology. By the late 1990s, a detailed understanding of V(D)J recombination mechanisms, found to be controlled by

Hydrodynamic cavitation (HC) and Fe(II) are advanced oxidation processes, in which pentachlorophenol (PCP) is treated by the redox method of activating persulfate (PS). The kinetics and mechanism of the HC and Fe(II) activation of PS were examined in aqueous solution using an electron spin resonance (ESR) spin trapping technique and radical trapping with pure compounds. The optimum ratio of Fe(II)/PS was 1:2, and the hydroxyl radical (HO ) and sulfate radical (SO4 −) generation rate were 5.56 mM h−1 and 8.62 μM h−1, respectively. The generation rate and Rct of HO and SO4 − at pH 3 and 50 °C in the Fe(II)/PS/HC system are 7584.6 μM h−1, 0.013 and 24.02 μM h−1, 3.95, respectively. The number of radicals was reduced as the pH increased, and it increased with increasing temperature. The PCP reaction rate constants was 4.39 × 10−2 min−1 at pH 3 and 50 °C. The activation energy was 10.68 kJ mol−1. In addition, the mechanism of PCP treatment in the Fe(II)/PS/HC system was a redox reaction, and the HO −/SO4 − contribution was 81.1 and 18.9%, respectively. In this study, we first examined PCP oxidation through HO and SO4 − quantification using only the Fe(II)/PS/HC process. Furthermore, the results provide the foundation for activation of PS by HC and Fe(II), but also provide a data basis for similar organic treatments other than PCP.

Immunoglobulin heavy chain (IgH) locus-associated G-rich long noncoding RNA (SμGLT) is important for physiological and pathological B cell DNA recombination. We demonstrate that the METTL3 enzyme-catalyzed N(6)-methyladenosine (m(6)A) RNA modification drives recognition and 3’-end processing of SμGLT by the RNA exosome, promoting class switch recombination (CSR) and suppressing chromosomal translocations. The recognition is driven by interaction of the MPP6 adaptor protein with nuclear m(6)A reader YTHDC1. MPP6 and YTHDC1 promote CSR by recruiting AID and the RNA exosome to actively transcribing SμGLT. Direct suppression of m(6)A modification of SμGLT or of m(6)A reader YTHDC1 reduces CSR. Moreover, METTL3, an essential gene for B cell development in the bone marrow and germinal center, suppresses IgHassociated aberrant DNA breaks and prevents genomic instability. Taken together, we propose coordinated and central roles for MPP6, m(6)A modification, and m(6)A reader proteins in controlling long noncoding RNA processing, DNA recombination and development in B cells.

RIPK1, RIPK3, ZBP1 and TRIF, the four mammalian proteins harboring RIP homotypic interaction motif (RHIM) domains, are key components of inflammatory signaling and programmed cell death. RHIM-domain protein activation is mediated by their oligomerization; however, mechanisms that promote a return to homeostasis remain unknown. Here we show that autophagy is critical for the turnover of all RHIM-domain proteins. Macrophages lacking the autophagy gene Atg16l1accumulated highly insoluble forms of RIPK1, RIPK3, TRIF and ZBP1. Defective autophagy enhanced necroptosis by Tumor necrosis factor (TNF) and Toll-like receptor (TLR) ligands. TNF-mediated necroptosis was mediated by RIPK1 kinase activity, whereas TLR3- or TLR4-mediated death was dependent on TRIF and RIPK3. Unexpectedly, combined deletion of Atg16l1 and Zbp1 accelerated LPS-mediated necroptosis and sepsis in mice. Thus, ZBP1 drives necroptosis in the absence of the RIPK1-RHIM, but suppresses this process when multiple RHIM-domain containing proteins accumulate. These findings identify autophagy as a central regulator of innate inflammation governed by RHIM-domain proteins.

SUMMARYMitochondrial dysfunction and proteostasis failure frequently coexist as hallmarks of neurodegenerative disease. How these pathologies are related is not well understood. Here we describe a phenomenon termed MISTERMINATE (mitochondrial stress-induced translational termination impairment and protein carboxyl terminal extension), which mechanistically links mitochondrial dysfunction with proteostasis failure. We show that mitochondrial dysfunction impairs translational termination of nuclear-encoded mitochondrial mRNAs including complex-I 30kD subunit (C-I30) mRNA, occurring on mitochondrial surface in Drosophila and mammalian cells. Ribosomes stalled at the normal stop codon continue to add to the C-terminus of C-I30 certain amino acids non-coded by mRNA template. C-terminally-extended C-I30 is toxic when assembled into C-I and forms aggregates in the cytosol. Enhancing co-translational quality control prevents C-I30 C-terminal extension and rescues mitochondrial and neuromuscular degeneration in a Parkinson’s disease model. These findings emphasize the importance of efficient translation termination and reveal unexpected link between mitochondrial health and proteome homeostasis mediated by MISTERMINATE.