IF 2.3 3区生物学

Prion Pub Date : 2020-12-01 DOI: 10.1080/19336896.2020.1812367

Yumeng Huang, Ma Jianfang, Rodrigo Morales, Huidong Tang

{"title":"Corticobasal manifestations of Creutzfeldt-Jakob disease with D178N-homozygous 129M genotype.","authors":"Yumeng Huang, Ma Jianfang, Rodrigo Morales, Huidong Tang","doi":"10.1080/19336896.2020.1812367","DOIUrl":"https://doi.org/10.1080/19336896.2020.1812367","url":null,"abstract":"Creutzfeldt-Jakob disease (CJD) is a prion disease, usually presented with memory loss, ataxia, dementia, myoclonus, involuntary movements and psychiatric problems. D178N-homozygous 129M genotype has been recognized in the diagnosis of fatal familial insomnia (FFI) globally. Here we report a patient presented with progressive left upper limb stiffness, bradykinesia, hypomimia and weight loss (10 kg) initially. She progressed to dementia, dysphasia, dysphonia and be bedridden quickly but did not present insomnia. She was diagnosed with CJD corticobasal subtype carrying a classic D178N-129M mutation of PRNP in FFI. Remarkably, she has a strong family history of neurological degeneration diseases but the other members of this pedigree who do not carry D178N-homozygous 129M mutation in PRNP do not present any CJD or FFI symptoms. We conclude that this patient carrying D178N-homozygous 129M mutation in PRNP should be diagnosed as CJD. Thus, the clinicopathology should be considered as a crucial evidence in diagnosing some cases, but FFI could be evaluated as a differential diagnosis with a unique clinical profile. List of abbreviations AD: Alzheimer disease; ADL: Activities of Daily Living; CBD Cortical basal degeneration; CBS: Corticobasal syndrome; CJD: Creutzfeldt-Jakob disease; DWI: Diffusion-weighted image; EEG: Electroencephalograph, fCJD: familial Creutzfeld-Jakob disease; FFI: Fatal familial insomnia; FLAIR: Fluid-attenuated inversion recovery; MMSE: Mini-mental state examination; MoCA: Montreal Cognitive Assessment; MRI: Magnetic resonance imaging; PD: Parkinson disease; PrP: Prion protein; PSWC: Periodic sharp wave complexes; SWI: Susceptibility-weighted imaging.","PeriodicalId":54585,"journal":{"name":"Prion","volume":null,"pages":null},"PeriodicalIF":2.3,"publicationDate":"2020-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1080/19336896.2020.1812367","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"38393517","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"生物学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 1

Rare genetic E196A mutation in a patient with Creutzfeldt-Jakob disease: a case report and literature. 克雅氏病罕见基因E196A突变1例报告及文献

IF 2.3 3区生物学

Prion Pub Date : 2020-12-01 DOI: 10.1080/19336896.2020.1769528

Xiping Wu, Zhao Cui, Xie Guomin, Haifeng Wang, Xiaoling Zhang, Zhiguang Li, Qi Sun, Feiteng Qi

{"title":"Rare genetic E196A mutation in a patient with Creutzfeldt-Jakob disease: a case report and literature.","authors":"Xiping Wu, Zhao Cui, Xie Guomin, Haifeng Wang, Xiaoling Zhang, Zhiguang Li, Qi Sun, Feiteng Qi","doi":"10.1080/19336896.2020.1769528","DOIUrl":"https://doi.org/10.1080/19336896.2020.1769528","url":null,"abstract":"Genetic Creutzfeldt-Jakob disease (gCJD) is characterized by mutations in the PRNP gene and represents approximately 10-15% of the human prion diseases. Here, we report a 42-year-old Chinese man who was diagnosed with gCJD. The patient had a rare mutation in codon 196 (E196A) of PRNP leading to an exchange of amino acid from glutamic acid (E) to alanine (A). The polymorphism of codon 129 in the patient was methionine homozygote. His mother and daughter are asymptomatic carriers of the same mutation. The clinical manifestations were similar to those of sporadic CJD. 14-3-3 protein was positive in cerebrospinal fluid, and there were sharp slow complex waves in electroencephalography and ribbon-like signals on magnetic resonance imaging (MRI). The main complaints of patient changed from visual space and visual colour to psychotic symptoms with enhanced high signal intensity on the occipital and frontal cortices on MRI. We compared the clinical characteristics of the current patient with those of previously reported Chinese patients with other gCJD of E196A mutation to summarize the common features of E196A gCJD.","PeriodicalId":54585,"journal":{"name":"Prion","volume":null,"pages":null},"PeriodicalIF":2.3,"publicationDate":"2020-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1080/19336896.2020.1769528","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"38014521","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"生物学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 5

Management of chronic wasting disease in ranched elk: conclusions from a longitudinal three-year study. 牧场麋鹿慢性消耗性疾病的管理:一项为期三年的纵向研究的结论。

IF 2.3 3区生物学

Prion Pub Date : 2020-12-01 DOI: 10.1080/19336896.2020.1724754

N J Haley, D M Henderson, R Donner, S Wyckoff, K Merrett, J Tennant, E A Hoover, D Love, E Kline, A D Lehmkuhl, B V Thomsen

{"title":"Management of chronic wasting disease in ranched elk: conclusions from a longitudinal three-year study.","authors":"N J Haley, D M Henderson, R Donner, S Wyckoff, K Merrett, J Tennant, E A Hoover, D Love, E Kline, A D Lehmkuhl, B V Thomsen","doi":"10.1080/19336896.2020.1724754","DOIUrl":"https://doi.org/10.1080/19336896.2020.1724754","url":null,"abstract":"Chronic wasting disease is a fatal, horizontally transmissible prion disease of cervid species that has been reported in free-ranging and farmed animals in North America, Scandinavia, and Korea. Like other prion diseases, CWD susceptibility is partly dependent on the sequence of the prion protein encoded by the host's PRNP gene; it is unknown if variations in PRNP have any meaningful effects on other aspects of health. Conventional diagnosis of CWD relies on ELISA or IHC testing of samples collected post-mortem, with recent efforts focused on antemortem testing approaches. We report on the conclusions of a study evaluating the role of antemortem testing of rectal biopsies collected from over 570 elk in a privately managed herd, and the results of both an amplification assay (RT-QuIC) and conventional IHC among animals with a several PRNP genotypes. Links between PRNP genotype and potential markers of evolutionary fitness, including pregnancy rates, body condition, and annual return rates were also examined. We found that the RT-QuIC assay identified significantly more CWD positive animals than conventional IHC across the course of the study, and was less affected by factors known to influence IHC sensitivity - including follicle count and PRNP genotype. We also found that several evolutionary markers of fitness were not adversely correlated with specific PRNP genotypes. While the financial burden of the disease in this herd was ultimately unsustainable for the herd owners, our scientific findings and the hurdles encountered will assist future CWD management strategies in both wild and farmed elk and deer.","PeriodicalId":54585,"journal":{"name":"Prion","volume":null,"pages":null},"PeriodicalIF":2.3,"publicationDate":"2020-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1080/19336896.2020.1724754","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10764522","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"生物学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 10

Serial evaluation of swallowing function in a long-term survivor of V180I genetic Creutzfeldt-Jakob disease. V180I遗传性克雅氏病长期存活患者吞咽功能的系列评价

IF 2.3 3区生物学

Prion Pub Date : 2020-12-01 DOI: 10.1080/19336896.2020.1787090

Kenjiro Kunieda, Yuichi Hayashi, Megumi Yamada, Masahiro Waza, Tomonori Yaguchi, Ichiro Fujishima, Takayoshi Shimohata

{"title":"Serial evaluation of swallowing function in a long-term survivor of V180I genetic Creutzfeldt-Jakob disease.","authors":"Kenjiro Kunieda, Yuichi Hayashi, Megumi Yamada, Masahiro Waza, Tomonori Yaguchi, Ichiro Fujishima, Takayoshi Shimohata","doi":"10.1080/19336896.2020.1787090","DOIUrl":"https://doi.org/10.1080/19336896.2020.1787090","url":null,"abstract":"Swallowing function in long-term survivors with Creutzfeldt-Jakob disease (CJD) remains unknown. Herein, we demonstrated serial evaluation of swallowing function in a case with V180I genetic CJD (gCJD) using videofluoroscopic examination of swallowing (VF). A 69-year-old woman was admitted to our hospital because of bradykinesia and memory disturbances 4 months after the onset of symptoms. Neurological examination revealed dementia, bradykinesia and frontal signs. Diffusion-weighted MRI revealed bilateral cortical hyperintensity in the frontal, temporal, and parietal cortices, and PRNP gene analysis indicated a V180I mutation. Her dysphagia gradually progressed, and she received percutaneous gastrostomy 42 months after the onset. VF was performed at 27, 31, 39, and 79 months after the onset. Although bolus transport from oral cavity to pharynx gradually worsened and initiation of the pharyngeal swallow was gradually delayed, the pharyngeal swallowing function was preserved even at 72 months after onset. MRI revealed no apparent atrophy of brainstem, and single photon emission computed tomography showed preserved regional cerebral blood flow in the brainstem. These findings suggest that the pathophysiology of dysphagia in a long-term survivor of V180I gCJD is that of pseudobulbar palsy, likely owing to preserved brainstem function even in the akinetic mutism state.","PeriodicalId":54585,"journal":{"name":"Prion","volume":null,"pages":null},"PeriodicalIF":2.3,"publicationDate":"2020-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1080/19336896.2020.1787090","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"38122959","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"生物学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 4

Effect of the fluorescent probes ThT and ANS on the mature amyloid fibrils. 荧光探针ThT和ANS对成熟淀粉样蛋白原纤维的影响。

IF 2.3 3区生物学

Prion Pub Date : 2020-12-01 DOI: 10.1080/19336896.2020.1720487

M I Sulatsky, A I Sulatskaya, O I Povarova, Iu A Antifeeva, I M Kuznetsova, K K Turoverov

{"title":"Effect of the fluorescent probes ThT and ANS on the mature amyloid fibrils.","authors":"M I Sulatsky, A I Sulatskaya, O I Povarova, Iu A Antifeeva, I M Kuznetsova, K K Turoverov","doi":"10.1080/19336896.2020.1720487","DOIUrl":"https://doi.org/10.1080/19336896.2020.1720487","url":null,"abstract":"Fluorescent probes thioflavin T (ThT) and 1-anilino-8-naphthalene sulfonate (ANS) are widely used to study amyloid fibrils that accumulate in the body of patients with serious diseases, such as Alzheimer's, Parkinson's, prion diseases, etc. However, the possible effect of these probes on amyloid fibrils is not well understood. In this work, we investigated the photophysical characteristics, structure, and morphology of mature amyloid fibrils formed from two model proteins, insulin and lysozyme, in the presence of ThT and ANS. It turned out that ANS affects the secondary structure of amyloids (shown for fibrils formed from insulin and lysozyme) and their fibers clusterization (valid for lysozyme fibrils), while ThT has no such effects. These results confirm the differences in the mechanisms of these dyes interaction with amyloid fibrils. Observed effect of ANS was explained by the electrostatic interactions between the dye molecule and cationic groups of amyloid-forming proteins (unlike hydrophobic binding of ThT) that induce amyloids conformational changes. This interaction leads to weakening repulsion between positive charges of amyloid fibrils and can promote their clusterization. It was shown that when fibrillogenesis conditions and, consequently, fibrils structure is changing, as well as during defragmentation of amyloids by ultrasonication, the influence of ANS to amyloids does not change, which indicates the universality of the detected effects. Based on the obtained results, it was concluded that ANS should be used cautiously for the study of amyloid fibrils, since this fluorescence probe have a direct effect on the object of study.","PeriodicalId":54585,"journal":{"name":"Prion","volume":null,"pages":null},"PeriodicalIF":2.3,"publicationDate":"2020-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1080/19336896.2020.1720487","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"37601796","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"生物学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 33

Association of chronic wasting disease susceptibility with prion protein variation in white-tailed deer (Odocoileus virginianus). 白尾鹿慢性消耗性疾病易感性与朊蛋白变异的关系

IF 2.3 3区生物学

Prion Pub Date : 2020-12-01 DOI: 10.1080/19336896.2020.1805288

Yasuko Ishida, Ting Tian, Adam L Brandt, Amy C Kelly, Paul Shelton, Alfred L Roca, Jan Novakofski, Nohra E Mateus-Pinilla

{"title":"Association of chronic wasting disease susceptibility with prion protein variation in white-tailed deer (Odocoileus virginianus).","authors":"Yasuko Ishida, Ting Tian, Adam L Brandt, Amy C Kelly, Paul Shelton, Alfred L Roca, Jan Novakofski, Nohra E Mateus-Pinilla","doi":"10.1080/19336896.2020.1805288","DOIUrl":"https://doi.org/10.1080/19336896.2020.1805288","url":null,"abstract":"Chronic wasting disease (CWD) is caused by prions, infectious proteinaceous particles, PrPCWD. We sequenced the PRNP gene of 2,899 white-tailed deer (WTD) from Illinois and southern Wisconsin, finding 38 haplotypes. Haplotypes A, B, D, E, G and 9 others encoded Q95G96S100N103A123Q226, designated 'PrP variant A.' Haplotype C and 4 other haplotypes encoded PrP 'variant C' (Q95S96S100N103A123Q226). Haplotype F and two other haplotypes encoded PrP 'variant F' (H95G96S100N103A123Q226). The association of CWD with encoded PrP variants was examined in 2,537 tested WTD from counties with CWD. Relative to PrP variant A, CWD susceptibility was lower in deer with PrP variant C (OR = 0.26, p < 0.001), and even lower in deer with PrP variant F (OR = 0.10, p < 0.0001). Susceptibility to CWD was highest in deer with both chromosomes encoding PrP variant A, lower with one copy encoding PrP variant A (OR = 0.25, p < 0.0001) and lowest in deer without PrP variant A (OR = 0.07, p < 0.0001). There appeared to be incomplete dominance for haplotypes encoding PrP variant C in reducing CWD susceptibility. Deer with both chromosomes encoding PrP variant F (FF) or one encoding PrP variant C and the other F (CF) were all CWD negative. Our results suggest that an increased population frequency of PrP variants C or F and a reduced frequency of PrP variant A may reduce the risk of CWD infection. Understanding the population and geographic distribution of PRNP polymorphisms may be a useful tool in CWD management.","PeriodicalId":54585,"journal":{"name":"Prion","volume":null,"pages":null},"PeriodicalIF":2.3,"publicationDate":"2020-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1080/19336896.2020.1805288","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"38301716","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"生物学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 10

Estimation of amyloid aggregate sizes with semi-denaturing detergent agarose gel electrophoresis and its limitations. 半变性洗涤剂琼脂糖凝胶电泳法测定淀粉样蛋白聚集体大小及其局限性。

IF 2.3 3区生物学

Prion Pub Date : 2020-12-01 DOI: 10.1080/19336896.2020.1751574

Polina B Drozdova, Yury A Barbitoff, Mikhail V Belousov, Rostislav K Skitchenko, Tatyana M Rogoza, Jeremy Y Leclercq, Andrey V Kajava, Andrew G Matveenko, Galina A Zhouravleva, Stanislav A Bondarev

{"title":"Estimation of amyloid aggregate sizes with semi-denaturing detergent agarose gel electrophoresis and its limitations.","authors":"Polina B Drozdova, Yury A Barbitoff, Mikhail V Belousov, Rostislav K Skitchenko, Tatyana M Rogoza, Jeremy Y Leclercq, Andrey V Kajava, Andrew G Matveenko, Galina A Zhouravleva, Stanislav A Bondarev","doi":"10.1080/19336896.2020.1751574","DOIUrl":"https://doi.org/10.1080/19336896.2020.1751574","url":null,"abstract":"Semi-denaturing detergent agarose gel electrophoresis (SDD-AGE) was proposed by Vitaly V. Kushnirov in the Michael D. Ter-Avanesyan's laboratory as a method to compare sizes of amyloid aggregates. Currently, this method is widely used for amyloid investigation, but mostly as a qualitative approach. In this work, we assessed the possibilities and limitations of the quantitative analysis of amyloid aggregate size distribution using SDD-AGE results. For this purpose, we used aggregates of two well-characterized yeast amyloid-forming proteins, Sup35 and Rnq1, and developed a protocol to standardize image analysis and process the result. A detailed investigation of factors that may affect the results of SDD-AGE revealed that both the cell lysis method and electrophoresis conditions can substantially affect the estimation of aggregate size. Despite this, quantitative analysis of SDD-AGE results is possible when one needs to estimate and compare the size of aggregates on the same gel, or even in different experiments, if the experimental conditions are tightly controlled and additional standards are used.","PeriodicalId":54585,"journal":{"name":"Prion","volume":null,"pages":null},"PeriodicalIF":2.3,"publicationDate":"2020-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1080/19336896.2020.1751574","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"37847681","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"生物学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 9

Analysis of Chinese patients with sporadic Creutzfeldt-Jakob disease. 中国散发性克雅氏病病例分析。

IF 2.3 3区生物学

Prion Pub Date : 2020-12-01 DOI: 10.1080/19336896.2020.1761515

Jing Yang, Haiyan Kuang, Qiong Wang, Jiao Liu, Xueping Chen, Huifang Shang

{"title":"Analysis of Chinese patients with sporadic Creutzfeldt-Jakob disease.","authors":"Jing Yang, Haiyan Kuang, Qiong Wang, Jiao Liu, Xueping Chen, Huifang Shang","doi":"10.1080/19336896.2020.1761515","DOIUrl":"https://doi.org/10.1080/19336896.2020.1761515","url":null,"abstract":"Sporadic Creutzfeldt-Jakob disease (sCJD) is a rare, incurable, and fatal neurodegenerative disorder. The objective of this study was to describe the clinical features and survival time of Chinese sCJD patients, and to explore the associations between clinical data and survival. In this study, we analysed the clinical data of 21 sCJD patients in a tertiary care hospital and used all Chinese case material available from 152 patients with sCJD in literatures between 2008 and 2018. The mean age of onset of all 173 deceased patients was 61.44 year-olds (y), with the highest incidence in the population of 60 to 69 y. The most common manifestation at disease onset was progressive dementia. With the progression of the disease, the four main clinical symptoms and signs were developed, including myoclonus, visual or cerebella disturbance, pyramidal or extrapyramidal dysfunction, and akinetic mutism. Extrapyramidal symptoms were more frequently observed. The mean survival time was 7.34 months, and 82.10% of cases died within 1 year after disease onset. The follow-up showed that the survival time was longer and the myoclonus sign was more frequently presented in younger-onset sCJD patients. Patients with abnormalities only in cortical regions had a higher frequency of pyramidal dysfunction than patients having lesions in both cortex and basal ganglia. The findings of this study might provide some insight into the clinical characteristics of sCJD patients in China, but further studies could examine the presences of clinical features and survival time in patients with early age of onset in a prospective manner.","PeriodicalId":54585,"journal":{"name":"Prion","volume":null,"pages":null},"PeriodicalIF":2.3,"publicationDate":"2020-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1080/19336896.2020.1761515","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"37909265","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"生物学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 4

An enigmatic case of cortical anopsia: Antemortem diagnosis of a 14-3-3 negative Heidenhain-variant MM1-sCJD. 一个神秘的皮质性失盲病例:14-3-3阴性Heidenhain-variant MM1-sCJD的死前诊断。

IF 2.3 3区生物学

Prion Pub Date : 2020-12-01 DOI: 10.1080/19336896.2019.1706703

Julius Obergassel, Lisa Lohmann, Sven G Meuth, Heinz Wiendl, Oliver Grauer, Christopher Nelke

引用次数: 3

Methionine oxidation within the prion protein. 朊蛋白内的蛋氨酸氧化。

IF 1.9 3区生物学

Prion Pub Date : 2020-12-01 DOI: 10.1080/19336896.2020.1796898

John Bettinger, Sina Ghaemmaghami

{"title":"Methionine oxidation within the prion protein.","authors":"John Bettinger, Sina Ghaemmaghami","doi":"10.1080/19336896.2020.1796898","DOIUrl":"10.1080/19336896.2020.1796898","url":null,"abstract":"Prion diseases are characterized by the self-templated misfolding of the cellular prion protein (PrPC) into infectious aggregates (PrPSc). The detailed molecular basis of the misfolding and aggregation of PrPC remains incompletely understood. It is believed that the transient misfolding of PrPC into partially structured intermediates precedes the formation of insoluble protein aggregates and is a critical component of the prion misfolding pathway. A number of environmental factors have been shown to induce the destabilization of PrPC and promote its initial misfolding. Recently, oxidative stress and reactive oxygen species (ROS) have emerged as one possible mechanism by which the destabilization of PrPC can be induced under physiological conditions. Methionine residues are uniquely vulnerable to oxidation by ROS and the formation of methionine sulfoxides leads to the misfolding and subsequent aggregation of PrPC. Here, we provide a review of the evidence for the oxidation of methionine residues in PrPC and its potential role in the formation of pathogenic prion aggregates.","PeriodicalId":54585,"journal":{"name":"Prion","volume":null,"pages":null},"PeriodicalIF":1.9,"publicationDate":"2020-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7518762/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"38229714","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"生物学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

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