Abstract:
:The pathogenic mechanism of prion diseases remains unknown. We recently reported that prion infection disturbs post-Golgi trafficking of certain types of membrane proteins to the cell surface, resulting in reduced surface expression of membrane proteins and abrogating the signal from the proteins. The surface expression of the membrane proteins was reduced in the brains of mice inoculated with prions, well before abnormal symptoms became evident. Prions or pathogenic prion proteins were mainly detected in endosomal compartments, being particularly abundant in recycling endosomes. Some newly synthesized membrane proteins are delivered to the surface from the Golgi apparatus through recycling endosomes, and some endocytosed membrane proteins are delivered back to the surface through recycling endosomes. These results suggest that prions might cause neuronal dysfunctions and cell loss by disturbing post-Golgi trafficking of membrane proteins via accumulation in recycling endosomes. Interestingly, it was recently shown that delivery of a calcium channel protein to the cell surface was impaired and its function was abrogated in a mouse model of hereditary prion disease. Taken together, these results suggest that impaired delivery of membrane proteins to the cell surface is a common pathogenic event in acquired and hereditary prion diseases.
journal_name
Prionjournal_title
Prionauthors
Uchiyama K,Miyata H,Sakaguchi Sdoi
10.4161/pri.27381subject
Has Abstractpub_date
2013-11-01 00:00:00pages
447-51issue
6eissn
1933-6896issn
1933-690Xpii
27381journal_volume
7pub_type
杂志文章相关文献
Prion文献大全abstract::The evolutionary origins of vertebrate prion genes had remained elusive until recently when multiple lines of evidence converged to the proposition that members of the prion gene family represent an ancient branch of a larger family of ZIP metal ion transporters. (1) A follow-up investigation which explored the mechan...
journal_title:Prion
pub_type: 杂志文章
doi:10.4161/pri.20196
更新日期:2012-09-01 00:00:00
abstract::Prion diseases are consistently associated with prion protein (PrP(C)) misfolding rendering a cascade of auto-catalytic self-perpetuation of misfolded PrP in an afflicted individual. The molecular process is intriguingly similar to all known amyloid diseases both local and systemic. The prion disease is also infectiou...
journal_title:Prion
pub_type: 杂志文章
doi:10.4161/pri.27601
更新日期:2014-01-01 00:00:00
abstract::The yeast prion [PSI(+)] represents an aggregated state of the translational release factor Sup35 (eRF3) and deprives termination complexes of functional Sup35, resulting in nonsense codon suppression. Protein-remodeling factor Hsp104 is involved in thermotolerance and [PSI(+)] propagation, however the structure-and-f...
journal_title:Prion
pub_type: 杂志文章
doi:10.4161/pri.1.1.4060
更新日期:2007-01-01 00:00:00
abstract::Transmissible spongiform encephalopathies (TSEs) are a group of neurodegenerative diseases caused by the misfolding of the cellular prion protein to an infectious form PrP(Sc). The intercellular transfer of PrP(Sc) is a question of immediate interest as the cell-to-cell movement of the infectious particle causes the i...
journal_title:Prion
pub_type: 杂志文章
doi:10.1080/19336896.2015.1025189
更新日期:2015-01-01 00:00:00
abstract::Mechanisms for the spread of transmissible spongiform encephalopathy diseases, including chronic wasting disease (CWD) in North American cervids, are incompletely understood, but primary routes include horizontal and environmental transmission. Birds have been identified as potential vectors for a number of diseases, ...
journal_title:Prion
pub_type: 杂志文章,评审
doi:10.4161/pri.25621
更新日期:2013-07-01 00:00:00
abstract::Modified nucleosides in tRNA anticodon loops such as 5-methoxy-carbonyl-methyl-2-thiouridine (mcm5s2U) and pseuduridine (Ψ) are thought to be required for an efficient decoding process. In Saccharomyces cerevisiae, the simultaneous presence of mcm5s2U and Ψ38 in tRNAGlnUUG was shown to mediate efficient synthesis of t...
journal_title:Prion
pub_type: 杂志文章
doi:10.1080/19336896.2017.1284734
更新日期:2017-01-02 00:00:00
abstract::Many neurodegenerative disorders share common features including the accumulation of aggregated misfolded proteins, neuroinflammation and the induction of apoptosis. While the contributions of each of these individual elements to neuronal death remain unclear, a commonly used antibiotic, minocycline, has been shown to...
journal_title:Prion
pub_type: 杂志文章,评审
doi:10.4161/pri.3.2.8820
更新日期:2009-04-01 00:00:00
abstract::Early detection and diagnosis of neurodegenerative diseases has been hampered by the lack of sensitive testing. Real-time quaking induced conversion (RT-QuIC) has been used for the early and sensitive detection of prion-induced neurologic disease, and has more recently been adapted to detect misfolded alpha-synuclein ...
journal_title:Prion
pub_type: 杂志文章
doi:10.1080/19336896.2020.1832946
更新日期:2020-12-01 00:00:00
abstract::Growing evidence indicates that astrocytes cannot be just considered as passive supportive cells deputed to preserve neuronal activity and survival, but rather they are involved in a striking number of active functions that are critical to the performance of the central nervous system (CNS). As a consequence, it is be...
journal_title:Prion
pub_type: 杂志文章,评审
doi:10.4161/pri.22512
更新日期:2013-01-01 00:00:00
abstract::The description of prions as causal agents of Transmissible Spongiform Encephalopathies (TSE), is nowadays accepted as an important breakthrough in biology as revealed the existence of a completely new group of pathogens and a new way of transmission for biological information. A common feature of many neurodegenerati...
journal_title:Prion
pub_type:
doi:10.1080/19336896.2019.1569451
更新日期:2019-01-01 00:00:00
abstract::Prion diseases are infectious conformational diseases. Despite the determination of many native prion protein (PrP) structures and in vitro production of infectious prions from recombinant PrP the structural background of PrP conversion remains the largest unsolved problem. The aggregated state of PrP (Sc) makes it in...
journal_title:Prion
pub_type: 杂志文章
doi:10.4161/pri.5.2.16232
更新日期:2011-04-01 00:00:00
abstract::Prions are self-propagating infectious protein aggregates of mammals and fungi. The exact mechanism of prion formation is poorly understood. In a recent study, a comparative analysis of the aggregation propensities of chimeric proteins derived from the yeast Sup35p and mouse PrP prion proteins was performed in neurobl...
journal_title:Prion
pub_type: 杂志文章
doi:10.4161/pri.2.3.7147
更新日期:2008-07-01 00:00:00
abstract::We report here on the proceedings of the Global Alzheimer Summit that took place September 22-23, 2011 in Madrid, Spain. As Alzheimer disease (AD) is the leading cause of neurodegeneration in elderly individuals and as yet has no effective therapeutic option, it continues to stimulate global research interests. At the...
journal_title:Prion
pub_type:
doi:10.4161/pri.6.1.18854
更新日期:2012-01-01 00:00:00
abstract::Prions, the causative agent of chronic wasting disease (CWD) enter the environment through shedding of bodily fluids and carcass decay, posing a disease risk as a result of their environmental persistence. Plants have the ability to take up large organic particles, including whole proteins, and microbes. This study us...
journal_title:Prion
pub_type: 杂志文章
doi:10.4161/pri.27963
更新日期:2014-01-01 00:00:00
abstract::The detection method based on the mathematical expectation (ME) strategy is fast and accuracy for low frequency mutation screening in large samples. Previous studies have found that the 14-bp insertion/deletion (indel) variants of the 3' untranslated region (3' UTR) within bovine PRNP gene have been characterized with...
journal_title:Prion
pub_type: 杂志文章
doi:10.1080/19336896.2016.1211593
更新日期:2016-09-02 00:00:00
abstract::M2B cells with persistent classical bovine spongiform encephalopathy (C-BSE) have been established previously. In this study, we performed strain characterization of the M2B cell line in bovine PrPC overexpressing mice (Tg 1896). Mice intracranially inoculated with M2B cells and C-BSE survived for 451 ± 7 and 465 ± 31...
journal_title:Prion
pub_type: 杂志文章
doi:10.1080/19336896.2017.1331809
更新日期:2017-11-02 00:00:00
abstract::The yeast, fungal and mammalian prions determine heritable and infectious traits that are encoded in alternative conformations of proteins. They cause lethal sporadic, familial and infectious neurodegenerative conditions in man, including Creutzfeldt-Jakob disease (CJD), Gerstmann-Sträussler-Scheinker syndrome (GSS), ...
journal_title:Prion
pub_type: 杂志文章,评审
doi:10.4161/pri.18666
更新日期:2012-04-01 00:00:00
abstract::A variety of signaling pathways, in particular with roles in cell fate and host defense, operate by a prion-like mechanism consisting in the formation of open-ended oligomeric signaling complexes termed signalosomes. This mechanism emerges as a novel paradigm in signal transduction. Among the proteins forming such sig...
journal_title:Prion
pub_type: 杂志文章,评审
doi:10.1080/19336896.2015.1038018
更新日期:2015-01-01 00:00:00
abstract::Here, we report a Chinese case of Creutzfeldt-Jakob disease (CJD) with a rare mutation in the prion protein gene (PRNP) leading to an exchange of amino acid from valine (Val) to isoleucine (I) at codon 203 (V203I). The 80-y-old male presented with sudden memory loss, rapid loss of vocabulary, inattention and slow resp...
journal_title:Prion
pub_type: 杂志文章
doi:10.4161/pri.24674
更新日期:2013-05-01 00:00:00
abstract::Each known abnormal prion protein (PrP (Sc) ) is considered to have a specific range and therefore the ability to infect some species and not others. Consequently, some species have been assumed to be prion disease resistant as no successful natural or experimental challenge infections have been reported. This assumpt...
journal_title:Prion
pub_type: 杂志文章
doi:10.4161/pri.22057
更新日期:2012-11-01 00:00:00
abstract::It has been described that the breakdown of β-sheets in PrP (Sc) by denaturation results in loss of infectivity and PK-sensitivity, suggesting a relationship between the structure and PK-resistance. It is also known that an important fraction of total PrP (Sc) is PK-sensitive and can be isolated by the method we alr...
journal_title:Prion
pub_type: 杂志文章
doi:10.4161/pri.22309
更新日期:2012-11-01 00:00:00
abstract::We propose models for in vitro grown mammalian prion protein fibrils based upon left handed beta helices formed both from the N-terminal and C-terminal regions of the proteinase resistant infectious prion core. The C-terminal threading onto a beta-helical structure is almost uniquely determined by fixing the cysteine ...
journal_title:Prion
pub_type: 杂志文章
doi:10.4161/pri.2.2.7059
更新日期:2008-04-01 00:00:00
abstract::Transmissible spongiform encephalopathies (TSEs) are a group of lethal neurodegenerative diseases involving the structural conversion of cellular prion protein (PrPC) into the pathogenic isoform (PrPSc) for which no effective treatment is currently available. Previous studies have implicated that a polymeric molecule ...
journal_title:Prion
pub_type: 杂志文章
doi:10.1080/19336896.2018.1505395
更新日期:2018-01-01 00:00:00
abstract::The aggregation of a soluble protein into insoluble, beta-sheet rich amyloid fibrils is a defining characteristic of many neurodegenerative diseases, including prion disorders. The prion protein has so far been considered unique because of its infectious nature. Recent investigations, however, suggest that other amylo...
journal_title:Prion
pub_type: 杂志文章,评审
doi:10.4161/pri.3.2.8754
更新日期:2009-04-01 00:00:00
abstract::Studies of the ovine prion-related protein (testis-specific) gene (PRNT), including studies of genetic diversity, have highlighted its potential relationship to scrapie infection and economically important ovine traits. PRNT was previously reported to be highly polymorphic in Portuguese sheep. To characterize genetic ...
journal_title:Prion
pub_type: 杂志文章
doi:10.1080/19336896.2018.1467193
更新日期:2018-01-01 00:00:00
abstract::We recently developed a new in vitro amplification technology, designated "real-time quaking-induced conversion (RT-QUIC)", for detection of the abnormal form of prion protein (PrPSc) in easily accessible specimens such as cerebrospinal fluid (CSF). After assessment of more than 200 CSF specimens from Japanese and Aus...
journal_title:Prion
pub_type: 杂志文章
doi:10.4161/pri.5.3.16893
更新日期:2011-07-01 00:00:00
abstract::Recent studies on iatrogenic Creutzfeldt-Jakob disease (CJD) raised concerns that one of the hallmark lesions of Alzheimer disease (AD), amyloid-β (Aβ), may be transmitted from human-to-human. The neuropathology of AD-related lesions is complex. Therefore, many aspects need to be considered in deciding on this issue. ...
journal_title:Prion
pub_type: 杂志文章,评审
doi:10.1080/19336896.2016.1228507
更新日期:2016-09-02 00:00:00
abstract::Synaptic dysfunction is a key process in the evolution of many neurodegenerative diseases, with synaptic loss preceding that of neuronal cell bodies. In Alzheimer, Huntington, and prion diseases early synaptic changes correlate with cognitive and motor decline, and altered synaptic function may also underlie deficits ...
journal_title:Prion
pub_type: 杂志文章,评审
doi:10.4161/pri.3.4.9981
更新日期:2009-10-01 00:00:00
abstract:OBJECTIVE:To evaluate whether EPC-MVs could promote bone regeneration by directly regulating osteoblast through miR-126. The underlying mechanisms were also explored. METHODS:EPCs were isolated from bone marrow mononuclear cells. EPC-MVs were collected from EPCs cultured medium. The lentivirus was used to induce miR-1...
journal_title:Prion
pub_type: 杂志文章
doi:10.1080/19336896.2019.1607464
更新日期:2019-01-01 00:00:00
abstract::Perturbations of calcium homeostasis have been associated with several neurodegenerative disorders. A common polymorphism (rs2986017) in the CALHM1 gene, coding for a regulator of calcium homeostasis, is a genetic risk factor for the development of Alzheimer disease (AD). Although some authors failed to confirm these ...
journal_title:Prion
pub_type: 杂志文章
doi:10.4161/pri.20785
更新日期:2012-09-01 00:00:00