Purpose:VISTA, an immune checkpoint enriched in microglia, regulates inflammatory signaling. Given microglial activation drives autoimmune uveitis, we investigated whether VISTA protects against experimental autoimmune uveitis (EAU) by modulating retinal microglia. Methods:VISTA expression was analyzed by flow cytometry in active VKH patients and healthy controls. Functional studies in LPS/IFN-γ-stimulated BV2 microglia used genetic knockdown/overexpression and modulating antibodies (13F3, MH5A). Activation status, cytokine secretion, migration, and TLR4/MyD88/NF-κB signaling were assessed. An EAU mouse model received intravitreal adeno-associated virus-mediated VISTA overexpression, with severity evaluated clinically and histopathologically. Results:VISTA was downregulated in circulating immune cells of VKH patients and in retinal microglia during EAU. In vitro, inflammatory stimuli reduced microglial VISTA. Its knockdown or blockade exacerbated microglial activation, pro-inflammatory mediator secretion (TNF-α, iNOS, COX2), and migration, while overexpression or agonism suppressed activation. Critically, intravitreal VISTA overexpression alleviated EAU severity. Mechanistically, VISTA deficiency potentiated activation by enhancing TLR4/MyD88/NF-κB signaling. Conclusions:VISTA is a crucial gatekeeper of ocular immune homeostasis. Its downregulation promotes uveitis via microglial TLR4/MyD88/NF-κB pathway activation, making VISTA signaling restoration a promising therapeutic strategy.

Parkinson's Disease (PD) is characterized by the aggregation and accumulation of α-synuclein (α-syn), along with abnormally high levels of α-syn phosphorylation at the serine 129 site (pSer 129 α-syn, p-α-syn). However, the mechanisms underlying the extensive phosphorylation at the serine 129 site in the pathogenesis of PD, as well as the role of p-α-syn in the process, remain unclear. Furthermore, though α-syn could bind to VAPB and loosen Endoplasmic Reticulum (ER)-mitochondria associations by disrupting VAPB-PTPIP51 tethers, whether and how the phosphorylation of α-syn at the serine 129 site regulates VAPB-PTPIP51 interactions, remains unclear. Herein, Co-Immunoprecipitation and Mass Spectrometry (CO-IP/MS) studies were preformed to identify and compare the Protein-Protein Interactions (PPIs) of phosphorylated and total α-syn in the midbrains of Thy1-SNCA transgenic mice. We further performed CO-IP and Molecular Dynamics (MD) simulation assays to confirm the influence of α-syn phosphorylation on the aforementioned interactions. Additionally, we performed Gene Ontology (GO) and Kyoto Encyclopedia of Genes and Genomes (KEGG) analyses to annotate the functional features of the common interacting proteins of p-α-syn and VAPB. The potential downstream proteins were further verified via CO-IP. According to the CO-IP and MD results, phosphorylation at the serine 129 site of α-syn increased VAPB-PTPIP51 interactions, and α-syn interacted directly with PTPIP51. Furthermore, functional and pathway enrichment analyses revealed that the common interacting proteins of p-α-syn and VAPB were significantly involved in protein binding, metal ion binding, structural constituent of the cytoskeleton, the intermediate filament cytoskeleton, and microtubule organization processes. Moreover, our findings confirmed the interactions of potential downstream target proteins (CLTC, CAMK2A, ATP1A3, and TUBB4B) with p-α-syn and VAPB. These findings collectively elucidate the structural underpinnings of serine 129 phosphorylation regulates the interaction between α-syn and both VAPB and PTPIP51. We hope that these findings will provide valuable insights into the role and regulatory mechanisms of serine 129 phosphorylation in the pathogenesis of pertinent diseases.

Objective To investigate the mechanisms underlying regional heterogeneity in the elevating patterns of palatal shelf during mammalian craniofacial development.Methods Using a mouse model of embryonic palatal development,we acquired coronal multi-plane slices of the palatal shelves before elevation(early E13.5),during elevation(late E13.5),and after elevation(early E14.5).Hematoxylin and eosin(HE)staining was performed to compare the morphological changes and spatial correlations between the palate and tongue.Immunofluorescence staining of myosin heavy chain 1(MYH1),a marker found in slow muscle fibers and responsible for muscle contraction and movement,was performed to observe the tongue muscle development characteristics at different stages.We also observed changes in the palatal shelf elevating patterns at early E13.5 in the absence of the tongue through HE-stained in vitro palate organ culture.Further immunofluorescence staining of tenascin-C,an extracellular matrix protein,was performed to evaluate the effect of the tongue on the elevating pattern of the palatal shelf along the anterior-posterior axis.Results HE staining results of the coronal multi-plane slices showed that during the elevation period,from the posterior toward anterior,the coronal height of the tongue decreased,lateral inclination and flattening increased,but the sagittal length of the tongue increased.The elevating pattern of the palatal shelf changed from slow remodeling to rapid flipping,and MYH1 was abundantly expressed in both the internal and external muscle bundles of the tongue during this period.According to findings from in vitro cultivation of palatal organs,the posterior part of the palatal shelf elevated without forming new lateral lingual protrusions in the absence of the tongue.The regional expression pattern of tenascin-C was consistent with that observed before elevation.The posterior palate exhibited an elevation pattern similar to that of the anterior region.Conclusion The tongue may play a crucial role in shaping the posterior morphological remodeling and distinct elevation patterns of the palatal shelf.

Retinal vascular diseases are typified by the proliferation of irregular and leaky microvessels, resulting in vision impairment. Although the etiology of retinal angiogenesis is not yet fully understood, it is evident that microglia play a pivotal role in promoting angiogenesis. Methods: In vivo, the METTL14 conditional knockout (cKO) mouse was constructed to investigate the role of METTL14 in oxygen-induced retinopathy (OIR). In vitro, a combination of methylated RNA immunoprecipitation sequencing (MeRIP-seq), RNA-sequencing (RNA-seq), RNA Immunoprecipitation (RIP) assay, dual-luciferase reporter assays, and Chromatin immunoprecipitation-qPCR (ChIP-qPCR), was performed to explore the underlying mechanisms. Results: The proteomic analysis of hypoxic microglia has uncovered a pronounced enrichment in pathways related to RNA modification. Western blot has revealed that N6-methyladenosine (m6A) methyltransferase-like 14 (METTL14) exhibits the most significant increase among the RNA methylases. METTL14 cKO mice within an OIR model showed fewer neovascular formations. Additionally, in co-culture with sh-METTL14 HMC3 cells, HRMECs also exhibited reduced angiogenesis capabilities. Mechanically, E3 ubiquitin-protein ligase BARD1 can directly interact with METTL14, leading to an up-regulation of METTL14 protein level in hypoxic microglia. METTL14 could directly modifies and regulates the transcription factor MAX Dimerization Protein 1 (MXD1), which is subsequently recognized by the m6A "reader" YTH domain-containing family protein 2 (YTHDF2). Consequently, the modified MXD1 modulates the expression of VEGFA and VCAM1, promotes retinal neovascularization. Conclusion: Our study highlights the critical role of METTL14 in the OIR model and suggests a novel therapeutic target for addressing retinal vascular diseases.

Phosphorylation of α-synuclein at serine-129 (p-α-syn) is a hallmark of Parkinson’s disease (PD) and constitutes nearly 90% of α-synuclein in Lewy bodies, playing a critical role in disease progression. Despite its pathological significance, the molecular targets and mechanisms driving p-α-syn-induced toxicity, particularly mitochondrial dysfunction, remain poorly understood. In this study, we observed mitochondrial dysfunction in primary cortical neurons derived from mice overexpressing human α-synuclein (h-α-syn), which also exhibit elevated levels of p-α-syn. Notably, inhibiting Ser129 phosphorylation improved mitochondrial function, underscoring the role of p-α-syn in mitochondrial damage. To investigate the molecular mechanism, we performed co-immunoprecipitation (CO-IP) combined with mass spectrometry (MS) to identify p-α-syn binding proteins. This analysis identified protein tyrosine phosphatase interacting protein 51 (PTPIP51) and vesicle-associated membrane protein-associated protein B (VAPB) as key binding partners. Both proteins are localized in the mitochondria-associated endoplasmic reticulum mem-brane (MAM) and essential for calcium transfer between the endoplasmic reticulum (ER) and mitochondria. Our results showed that p-α-syn binds to PTPIP51 and VAPB, disrupting calcium signaling between the ER and mitochondria. Importantly, inhibition of Ser129 phosphorylation partially rescued calcium homeostasis. These findings uncover a novel mechanism by which p-α-syn drives mitochondrial dysfunction and calcium dysregulation through its interactions with MAM-associated proteins, providing new insights into its role in PD pathogenesis and potential therapeutic targets.

α-Synuclein (α-syn) can form oligomers, protofibrils, and fibrils, which are associated with the pathogenesis of Parkinson’s disease and other synucleinopathies. Both the lipid peroxidation product 4-oxo-2-nonenal (ONE) and agitation can induce aggregation of α-syn and phosphorylated α-syn. Thus, clarification of the characteristics of different α-syn species could help to select suitable aggregates for diagnosis and elucidate the pathogenesis of diseases. Here, we characterized ONE-induced wild-type (WT) α-syn aggregates (OW), ONE-induced phosphorylated α-syn (p-α-syn) aggregates (OP), agitation-induced α-syn preformed fibrils (PFF), and agitation-induced p-α-syn preformed fibrils (pPFF). Thioflavin T (ThT) dying demonstrated that OW and OP had fewer fibrils than the PFF and pPFF. Transmission electron microscopy revealed that the lengths of PFF and pPFF were similar, but the diameters differed. OW and OP had more compact structures than PFF and pPFF. Aggregation of p-α-syn was significantly faster than WT α-syn. Furthermore, OW and OP were more sodium dodecyl sulfate-stable and proteinase K-resistant, suggesting greater stability and compactness, while aggregates of PFF and pPFF were more sensitive to proteinase K treatment. Both ONE- and agitation-induced aggregates were cytotoxic when added exogenously to SH-SY5Y cells with increasing incubation times, but the agitation-induced aggregates caused cell toxicity in a shorter time and more p-α-syn inclusions. Similarly, p-proteins were more cytotoxic than non-p-proteins. Finally, all four aggregates were used as standard antigens to establish sandwich enzyme-linked immunosorbent assay (ELISA). The results showed that the recognition efficiency of OW and OP was more sensitive than that of PFF and pPFF. The OW- and OP-specific ELISA for detection of p-α-syn and α-syn in plasma samples of Thy1-α-syn transgenic mice showed that the content of aggregates could reflect the extent of disease. ONE and agitation induced the formation of α-syn aggregates with distinct biophysical properties and biomedical applications.

AbstractBackgroundCerebral ischemic disease is a common cerebrovascular disease, especially ischemic stroke. Exercise has protective functions on brain tissues following cerebral ischemia‐reperfusion injury (CIRI), but its preventive effects and mechanisms in CIRI remain unclear. We aimed to investigate the effects and mechanisms of exercise preconditioning on CIRI.MethodsThe middle cerebral artery occlusion (MCAO) operation was prepared to establish CIRI rats. All rats were randomized into the MCAO, exercise (exercise preconditioning plus MCAO operation), vector (exercise preconditioning, MCAO operation plus intraventricular injection of empty vector), and tissue inhibitor of metalloprotease 1 overexpression (OE‐TIMP1, exercise preconditioning, MCAO operation plus intraventricular injection of OE‐TIMP1) groups.ResultsThe results indicated that exercise preconditioning suppressed approximately 66.67% of neurological deficit scores and 73.79% of TIMP1 mRNA expression in MCAO rats, which were partially offset by OE‐TIMP1. The protective effects of exercise against neuron death status and cerebral infarction size in MCAO rats were reversed by OE‐TIMP1. It also confirmed that exercise weakened apoptosis and oxidative stress damage, with notable increases of B‐cell lymphoma‐2, superoxide dismutase, and glutathione peroxidase production, and evident decreases of BCL2‐associated X, caspase 3, and malondialdehyde in MCAO rats, while these effects were partially reversed by OE‐TIMP1. Additionally, the inhibitory effects of exercise on the protein levels of TIMP1, hypoxia‐inducible factor‐alpha, vascular endothelial growth factor receptor 2, vascular endothelial growth factor, and neurogenic locus notch homolog protein 1 in MCAO rats were partially reversed by OE‐TIMP1.ConclusionAltogether, exercise preconditioning had protective effects on CIRI by restraining TIMP1, which provided new therapeutic strategies for preventing CIRI.

The accumulation of α-synuclein (α-syn), a key protein in Parkinson's disease (PD), contributes to progressive neuronal damage associated with mitochondrial dysfunction and interactions with various proteins. However, the precise mechanism by which α-syn affects energy metabolism remains unclear. In our study, we used human α-syn (hα-syn) transgenic mice, which exhibit progressive neuronal decline. Through an immunoprecipitation assay specific to hα-syn, we identified an enzyme in the mitochondrial tricarboxylic acid (TCA) cycle as a binding partner—mitochondrial aconitase 2 (ACO2), which converts citrate to isocitrate. Hα-syn increasingly interacted with ACO2 in mitochondria as mice aged, correlating with a progressive decrease in ACO2 activity. The overexpression of ACO2 and the addition of isocitrate, a downstream metabolite of ACO2, were observed to alleviate hα-syn-induced mitochondrial dysfunction and cytotoxicity. Furthermore, we designed an interfering peptide to block the interaction between ACO2 and hα-syn, which showed therapeutic effects in reducing hα-syn toxicity in vitro and in vivo. Our research establishes a direct link between α-syn and the TCA cycle and identifies ACO2 as a promising therapeutic target for improving mitochondrial function and reducing α-syn neurotoxicity in PD.

Sleep disturbances are commonly non-motor symptoms in Parkinson's diseases (PD). However, standard dopamine replacement therapies for the treatment of motor symptoms often prove inadequate in combating sleep disturbances. Previous studies conducted by our research group have reported the neuroprotective effects of tenuigenin, a natural extract from Polygala tenuifolia root, which has been traditionally employed in treating insomnia. The objective of this study was to investigate the potential of tenuigenin in modulating sleep-wake behaviors and elucidate the underlying mechanisms. We employed EEG/EMG recordings to evaluate the impact of tenuigenin on sleep-wake profiles. Furthermore, we utilized c-Fos immunostaining, whole-cell patch clamping and local field potentials (LFP) recording to explore the mechanisms involved in sleep-promoting effects of tenuigenin. Additionally, we examined the effects of tenuigenin on sleep-promoting in MPTP PD mice. Here, we found tenuigenin demonstrated a significant increase in NREM sleep and a reduction in sleep latency in mice, without altering the EEG power density. Moreover, tenuigenin increased c-Fos expression in the ventrolateral preoptic area (VLPO) and stimulated sleep-promoting neurons in VLPO. The sleep-promoting effects of tenuigenin were abolished when mice were pretreated with flumazenil, an antagonist at the benzodiazepine site of the GABAA receptor. Furthermore, tenuigenin was found to ameliorate sleep disturbances in MPTP-induced mice. The results suggesting that tenuigenin facilitated a type of NREM sleep comparable to physiological NREM sleep through interaction with the GABAA receptor. Additionally, tenuigenin demonstrated improvements in sleep disturbances in MPTP-induced PD mice, suggesting its potential as a sleep-promoting substance, particularly for PD patients experiencing sleep disturbances.

Synucleinopathies such as Parkinson’s disease, dementia with Lewy bodies and multiple system atrophy are characteristic for α -synuclein aggregates in neurons or glia, and are always manifested olfaction deficits at their primary onsets. It remains elusive why aggregation of α -synuclein predominantly affect the olfactory system. Employing the knockout mice, we investigate the physiological function of α-synuclein in olfactory system. We found that deletion of α-synuclein primarily interferes the projection of olfactory sensory neurons. iTRAQ based LC-MS identified that 188 proteins are differentially expressed, including 9 that were associated with axon guidance. Among them, NCK2 is most significantly down-regulated, which was indicated to be involved a PPI network of 21 proteins, including 11 players of the Ephrin receptor signaling pathway. Either α-synuclein deletion or NCK2 deficiency can inactivate Eph A4 receptor. Re-expressing α-synuclein in the α-synuclein knockout neurons reverse the NCK2, as well as the phosphorylated Eph A4 (the activated Eph A4). Thus, α-synuclein regulates axon guidance through NCK2-Eph A4 signaling pathway. Malfunction of α-synuclein, whether because of deletion or aggregation, may cause aberrant olfactory neurons projection and subsequent olfaction deficits. This extended our knowledge of effects of α-synuclein in olfactory system, which may explain why olfaction is usually impaired in some synucleinopathy related disorders such as Parkinson’s disease.

Mutations in PARK7 and the resulting alterations in its production protein (DJ-1) are tightly associated with Parkinson's disease. We generated a human induced pluripotent stem cell (iPSC) line (CIBi013-A) from a patient with young-onset Parkinson's disease (YOPD) who carried a novel homozygous PARK7 (DJ-1) mutation (chr1:8037723, c.334C>G). The generated iPSCs will be used for investigating phenotype and underlying molecular mechanisms in patient-derived cells.

Olfactory impairment is an initial non-motor symptom of Parkinson's disease that causes the deposition of aggregated α-synuclein (α-syn) in olfactory neurons. Transient receptor potential canonical (TRPC) channels are a diverse group of non-selective Ca2+ entry channels involved in the progression or pathogenesis of PD via Ca2+ homeostatic regulation. However, the relationship between TRPC and α-syn pathology in an olfactory system remains unclear. To address this issue, we assessed the olfactory function in α-syn transgenic mice. In contrast with control mice, the transgenic mice exhibited impaired olfaction, TRPC3 activation and apoptotic neuronal cell death in the olfactory system. Similar results were observed in primary cultures of olfactory neurons, that is TRPC3 activation, increasing intracellular Ca2+ concentration and apoptotic cell death in the α-syn-overexpressed neurons. These changes were significantly attenuated by TRPC3 knockdown. Therefore, our findings suggest that TRPC3 activation and calcium dyshomeostasis play a key role in α-syn-induced olfactory dysfunction in mice.

Objective:To explore the effect of observation, teaching and discussion (OTD) teaching in the training of junior nurses in Ophthalmology Department.Methods:From January 2018 to January 2020, convenience sampling was used to select 102 junior nurses in the Ophthalmology Department of Beijing Tongren Hospital affiliated to Capital Medical University as the research subject. The 49 nurses who received traditional teaching training from January 2018 to January 2019 were selected as the control group, and the 53 nurses who received OTD teaching training from February 2019 to January 2020 were selected as the observation group. The self-directed learning ability and critical thinking ability of the two groups of nurses were compared.Results:The total score and each dimension of the Chinese version of the Self-Rating Scale of Self-Directed Learning for nurses in the observation group, and the total score and each dimension score of the Critical Thinking Disposition Inventory-Chinese Version were higher than those in the control group, and the differences were statistically significant ( P<0.05) . Conclusions:OTD teaching can improve the self-directed learning ability and critical thinking ability of junior nurses in Ophthalmology Department, which is worthy of popularization and application.

The Lewy bodies (LBs) are the pathological hallmark of Parkinson's disease (PD). More than 90% of α-synuclein (α-syn) within LBs is phosphorylated at the serine-129 residue [pSer129 α-syn (p-α-syn)]. Although various studies have revealed that this abnormally elevated p-α-syn acts as a pathological biomarker and is involved in the pathogenic process of PD, the exact pathophysiological mechanisms of p-α-syn are still not fully understood. Therefore, the development of specific and reliable tools for p-α-syn detection is important. In this study, we generated a novel p-α-syn mouse monoclonal antibody (C140S) using hybridoma technology. To further identify the characteristics of C140S, we performed several in vitro assays using recombinant proteins, along with ex vivo assays utilizing the brains of Thy1-SNCA transgenic (Tg) mice, the preformed fibril (PFF)-treated neurons, and the brain sections of patients with PD. Our C140S specifically recognized human and mouse p-α-syn proteins both in vitro and ex vivo, and similar to commercial p-α-syn antibodies, the C140S detected higher levels of p-α-syn in the midbrain of the Tg mice. Using immunogold electron microscopy, these p-α-syn particles were partly deposited in the cytoplasm and colocalized with the outer mitochondrial membrane. In addition, the C140S recognized p-α-syn pathologies in the PFF-treated neurons and the amygdala of patients with PD. Overall, the C140S antibody was a specific and potential research tool in the detection and mechanistic studies of pathogenic p-α-syn in PD and related synucleinopathies.

目的 探索新冠肺炎疫情与"互联网+"医疗的开展对儿科线下就医门诊量的影响.方法 选择全国门诊量最大的儿科综合医院——首都医科大学附属北京儿童医院为研究对象,收集2018年至2021年首都医科大学附属北京儿童医院门诊量较大的代表性科室(皮肤科、耳鼻咽喉头颈外科、眼科等10个科室)线下门诊的月别门诊量,采用中断时间序列(interrupted time series,ITS)方法分析各科室门诊量的变化趋势.结果 新冠肺炎疫情发生后,耳鼻咽喉头颈外科(-6649.00,P<0.05)、中医科(-2778.29,P<0.05)、血液肿瘤中心(-2490.26,P<0.05)、呼吸科(-2297.02,P<0.05)、神经科(-2288.39,P<0.05)、消化科(-1877.28,P<0.05)的瞬时门诊量显著降低(P<0.05);在疫情影响的2020年2月至2021年7月之间,除保健中心的月门诊量呈现逐渐上升趋势(125.69,P<0.05),其他科室趋势不变(P>0.05)."互联网+"医疗实施9个月后,眼科(-780.00,P<0.05)、保健中心(-389.71,P<0.05)、神经科(-370.70,P<0.05)、风湿免疫科(-69.72,P<0.05)的线下门诊量变化呈逐月下降趋势;"互联网+"医疗开展后较疫情中未开展时期,门诊量增长速度有所减缓的科室分别为眼科(-1005.92,P<0.05)、保健中心(-515.41,P<0.05)、神经科(-384.50,P<0.05).结论 新冠肺炎疫情的爆发对于儿科线下诊疗造成了极大的影响,即时门诊量显著降低."互联网+"医疗的开展对于缓解线下门诊诊疗压力的效果初显,在长远效果方面可以起到持续分流线下门诊量的作用.

Parkinson's disease (PD) is the second most common chronic progressive neurodegenerative disease. The main pathological features are progressive degeneration of neurons and abnormal accumulation of α-synuclein. At present, the pathogenesis of PD is not completely clear, and many changes in the intestinal tract may be the early pathogenic factors of PD. These changes affect the central nervous system (CNS) through both nervous and humoral pathways. α-Synuclein deposited in the intestinal nerve migrates upward along the vagus nerve to the brain. Inflammation and immune regulation mediated by intestinal immune cells may be involved, affecting the CNS through local blood circulation. In addition, microorganisms and their metabolites may also affect the progression of PD. Therefore, paying attention to the multiple changes in the intestinal tract may provide new insight for the early diagnosis and treatment of PD.

AIMS:The etiology of Parkinson's disease (PD) is complex and the mechanism is unclear. It has become a top priority to find common factors that induce and affect PD pathology. We explored the key role of hypoxia in promoting the pathological propagation of α-synuclein (α-syn) and the progression of PD.METHODS:We performed PD modeling by conducting intracranial stereotaxic surgery in the unilateral striatum of mice. We then measured protein aggregation in vitro. The rotarod and pole tests were employed next to measure the damage of the phenotype. Pathological deposition and autophagy were also observed by immunofluorescence staining and protein levels measured by western blotting.RESULTS:We demonstrated that short-term hypoxia activated phosphorylated (p)-α-syn in mice. We confirmed that p-α-syn was more readily formed aggregates than α-syn in vitro. Furthermore, we found that hypoxia promoted the activation and propagation of endogenous α-syn, contributing to the earlier degeneration of dopaminergic neurons in the substantia nigra and the deposition of p-α-syn in our animal model. Finally, autophagy inhibition contributed to the above pathologies.CONCLUSION:Hypoxia was shown to accelerate the pathological progression and damage phenotype in PD model mice. The results provided a promising research target for determining common interventions for PD in the future.

蛋白质合成是一个复杂的过程.在特定情况下,翻译过程中会发生异常核糖体停滞导致无法有效回收核糖体及翻译的元件,从而影响细胞内正常的转录效率.同时,异常翻译的新生多肽通过积累聚集而扰乱蛋白质稳态环境,进而导致疾病的发生.核糖体翻译质量控制(ribosome associ-ated protein quality control pathway,RQC)为真核细胞核糖体回收、降解错误的新生多肽提供一条拯救途径.最新研究表明,线粒体表面也存在RQC调控,称为线粒体RQC(mitochondrial ribosome as-sociated protein quality control,mitoRQC).线粒体是真核细胞内参与能量生成和物质代谢的重要细胞器.线粒体中超过98％蛋白质是由核基因编码的,在细胞质中合成后运输到线粒体内,这些蛋白质可能会受到mitoRQC的调控.mitoRQC与线粒体内部调控机制共同维持线粒体的稳定性.阐明线粒体蛋白质翻译的调控机制对研究人类线粒体疾病等方面具有重要意义.本文将重点讨论RQC系统和mitoRQC系统的功能及与疾病的相关性.