Stress granules in the spinal muscular atrophy and amyotrophic lateral sclerosis: The correlation and promising therapy

Hu, Lidan; Mao, Shanshan; Lin, Li; Bai, Guannan; Liu, Bingjie; Mao, Jianhua

doi:10.1016/j.nbd.2022.105749

Cited by 9 publications

(5 citation statements)

References 202 publications

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“…With the increased expression of the SG marker proteins G3BP1 and TIA-1, the degree of liver injury, HIF-1α and apoptosis induced by acute liver failure decreases ( 162 ). In addition, Hu et al ( 163 ) found that impaired SGs are important in the pathogenesis of spinal muscular atrophy.…”

Section: Hypoxia-induced Biomolecular Condensates Assemblymentioning

confidence: 99%

Protein aggregation and biomolecular condensation in hypoxic environments (Review)

Li,

Hao,

Yang

et al. 2024

Int J Mol Med

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Due to molecular forces, biomacromolecules assemble into liquid condensates or solid aggregates, and their corresponding formation and dissolution processes are controlled. Protein homeostasis is disrupted by increasing age or environmental stress, leading to irreversible protein aggregation. Hypoxic pressure is an important factor in this process, and uncontrolled protein aggregation has been widely observed in hypoxia-related conditions such as neurodegenerative disease, cardiovascular disease, hypoxic brain injury and cancer. Biomolecular condensates are also high-order complexes assembled from macromolecules. Although they exist in different phase from protein aggregates, they are in dynamic balance under certain conditions, and their activation or assembly are considered as important regulatory processes in cell survival with hypoxic pressure. Therefore, a better understanding of the relationship between hypoxic stress, protein aggregation and biomolecular condensation will bring marked benefits in the clinical treatment of various diseases. The aim of the present review was to summarize the underlying mechanisms of aggregate assembly and dissolution induced by hypoxic conditions, and address recent breakthroughs in understanding the role of aggregates in hypoxic-related diseases, given the hypotheses that hypoxia induces macromolecular assemblage changes from a liquid to a solid phase, and that adenosine triphosphate depletion and ATP-driven inactivation of multiple protein chaperones play important roles among the process. Moreover, it is anticipated that an improved understanding of the adaptation in hypoxic environments could extend the overall survival of patients and provide new strategies for hypoxic-related diseases.

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Section: Hypoxia-induced Biomolecular Condensates Assemblymentioning

confidence: 99%

Protein aggregation and biomolecular condensation in hypoxic environments (Review)

Li,

Hao,

Yang

et al. 2024

Int J Mol Med

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“…Moreover, in some pathological conditions, such as hypoxia, a common feature of numerous pathological conditions, including myocardial infarction, stroke, inflammation, and malignant tumors, SG formation may inhibit cell apoptosis through translation arrest, prevention of unfolded proteins accumulation (Arimoto et al, 2008). Besides, SGs share many components with neuronal granules in neurons, which clearly indicates that SGs affect neurodegenerative diseases, amyotrophic lateral sclerosis (ALS) disease, frontotemporal lobar degeneration (FTLD), Alzheimer's disease (AD) and Spinal muscular atrophy (SMA; Anderson et al, 2015;Brownsword and Locker, 2022;Hu et al, 2022).…”

Section: The Dynamic Processes Of Sgsmentioning

confidence: 99%

“…Abnormal metabolism of SGs has been found in a variety of diseases, including but not limited to cancer, neurodegenerative diseases (NDs), viral infections, autoimmune disease, cataracts, glaucoma, diabetes, and brain ischemia (Moujaber et al, 2017). Given that SGs have drawn widespread concern in recent years, the correlations between cancer or NDs and SGs have already been widely described (Chen and Liu, 2017;Gao et al, 2019;Hu et al, 2022), while reviews about the role of SGs in viral infections are less understood. Some studies point out that viral infections can trigger a series of cellular stress reactions and consequently regulate the assembly or disassembly of SGs (McInerney et al, 2005), suggesting the importance of SGs in balancing the translation of host-and virusencoded mRNAs (Reineke and Neilson, 2019;Eiermann et al, 2020).…”

Section: Introductionmentioning

confidence: 99%

Multiple functions of stress granules in viral infection at a glance

Guan

Wang²,

Fu³

et al. 2023

Front. Microbiol.

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Stress granules (SGs) are distinct RNA granules induced by various stresses, which are evolutionarily conserved across species. In general, SGs act as a conservative and essential self-protection mechanism during stress responses. Viruses have a long evolutionary history and viral infections can trigger a series of cellular stress responses, which may interact with SG formation. Targeting SGs is believed as one of the critical and conservative measures for viruses to tackle the inhibition of host cells. In this systematic review, we have summarized the role of SGs in viral infection and categorized their relationships into three tables, with a particular focus on Severe Acute Respiratory Syndrome Coronavirus 2 (SARS-CoV-2) infection. Moreover, we have outlined several kinds of drugs targeting SGs according to different pathways, most of which are potentially effective against SARS-CoV-2. We believe this review would offer a new view for the researchers and clinicians to attempt to develop more efficacious treatments for virus infection, particularly for the treatment of SARS-CoV-2 infection.

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“…Alzheimer's disease, Parkinson's disease, Huntington's disease, and amyotrophic lateral sclerosis (ALS) are just a few examples of the many faces of NDs. The rising number of ND cases puts immense pressure on healthcare systems, families, and communities [6][7][8][9][10]. While the specific causes of each NDs vary, researchers have identified several key factors that contribute to their development, these include oxidative stress (an imbalance between free radicals and antioxidants) leading to cellular damage, protein aggregation (the abnormal buildup of misfolded proteins in the brain, forming toxic clumps), neuro-inflammation (chronic inflammation in the nervous system damaging brain cells), neurotransmission impairment (disruption of chemical communication between neurons), mitochondrial dysfunction (impaired energy production in brain cells), excitotoxicity (excessive stimulation of brain cells, leading to their death) [11].…”

Section: Introductionmentioning

confidence: 99%

The Preventive Potential of the Mycelial Submerged Culture of Ophiocordyceps sinensis against Neurodegenerative Diseases and Its Possible Active Compounds

Huang,

Shi,

et al. 2024

Preprint

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The pharmacological effects of the fruiting body of Ophiocordyceps sinensis (Os) exerted antioxidant, anti-virus, and immunomodulatory, whereas the anti-inflammatory effects and active components of Os mycelial submerged culture (OMSC) are not known. This study aimed to investigate the active compounds in the fermented liquid (FLOS), hot water (WEOS), and 50-95% (EEOS-50, EEOS-95) ethanol extracts of OMSC and their anti-inflammatory effects and potential mechanisms in LPS-stimulated microglial BV2 cells. The results demonstrated that all of the OMSC extracts could inhibit NO production in BV2 cells. EEOS-95 exhibited the strongest inhibitory effects (41 % inhibitory ability at 500 µg/mL), and its ergosterol, GABA, total phenolic, and total flavonoid contents were significantly higher than those of the other extracts (18.60, 18.60, 2.28, and 2.14 mg/g, p &lt; 0.05, respectively). EEOS-95 also has a strong inhibitory ability against IL-6, IL-1β and TNF-α with IC50 of 617, 277 and 507 µg/mL, respectively, which is higher than that of 1 mM melatonin. The anti-inflammatory mechanism of EEOS-95 seems to be associated with up-regulation of PPAR-γ/Nrf-2/HO-1 antioxidant-related expression and down-regulation of NF-κB/COX-2/iNOS pro-inflammatory expression signaling. Therefore, we hypothesized that EEOS-95 can prevent neurodegenerative diseases, but the effect on the application in vivo should be further investigated.

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Stress granules in the spinal muscular atrophy and amyotrophic lateral sclerosis: The correlation and promising therapy

Cited by 9 publications

References 202 publications

Protein aggregation and biomolecular condensation in hypoxic environments (Review)

Protein aggregation and biomolecular condensation in hypoxic environments (Review)

Multiple functions of stress granules in viral infection at a glance

The Preventive Potential of the Mycelial Submerged Culture of Ophiocordyceps sinensis against Neurodegenerative Diseases and Its Possible Active Compounds

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