解剖学和形态学
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听力与言语-语言病理学
行为科学
心脏和心血管系统
细胞和组织工程学
临床神经病学
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牙科,口腔外科和医学
皮肤病学
急诊医学
内分泌学和新陈代谢
肠胃学和肝脏学
老人病学和老年医学
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药理学和药剂学
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泌尿学和肾脏学
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心理学,临床
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
abstract::Prion diseases are subacute neurodegenerative diseases that affect humans and a range of domestic and free-ranging animal species. These diseases are characterized by the accumulation of PrP (Sc), an abnormally folded isoform of the cellular prion protein (PrP (C)), in affected tissues. The pathology during prion dise...
journal_title:Prion
pub_type: 杂志文章
doi:10.4161/pri.20676
更新日期:2012-09-01 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::In Transmissible Spongiform Encephalopathies (TSEs) and Alzheimer disease (AD) both misfolding and aggregation of specific proteins represent key features. Recently, it was observed that PrP (c) is a mediator of a synaptic dysfunction induced by Aβ oligomers. We tested a novel γ secretase modulator (CHF5074) in a mur...
journal_title:Prion
pub_type: 杂志文章
doi:10.4161/pri.6.1.18317
更新日期:2012-01-01 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 are infectious, self-propagating protein conformations. [PSI+], [RNQ+] and [URE3] are well characterized prions in Saccharomyces cerevisiae and represent the aggregated states of the translation termination factor Sup35, a functionally unknown protein Rnq1, and a regulator of nitrogen metabolism Ure2, respectiv...
journal_title:Prion
pub_type: 杂志文章,评审
doi:10.4161/pri.18307
更新日期:2011-10-01 00:00:00
abstract::Yeast have been extensively used to model aspects of protein folding diseases, yielding novel mechanistic insights and identifying promising candidate therapeutic targets. In particular, the neurodegenerative disorder Huntington disease (HD), which is caused by the abnormal expansion of a polyglutamine tract in the hu...
journal_title:Prion
pub_type: 杂志文章,评审
doi:10.4161/pri.18005
更新日期:2011-10-01 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::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::Amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration (FTLD) are clinically overlapping neurodegenerative disorders whose pathophysiology remains incompletely understood. ALS initiates in a discrete location, and typically progresses in a pattern consistent with spread of the degenerative process t...
journal_title:Prion
pub_type: 杂志文章,评审
doi:10.4161/pri.5.1.14265
更新日期:2011-01-01 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::Prions are responsible for a heterogeneous group of fatal neurodegenerative diseases. They can be sporadic, genetic, or infectious disorders involving post-translational modifications of the cellular prion protein (PrP(C)). Prions (PrP(Sc)) are characterized by their infectious property and intrinsic ability to conver...
journal_title:Prion
pub_type: 杂志文章,评审
doi:10.4161/pri.3.4.10181
更新日期:2009-10-01 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::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::Scrapie and CWD are horizontally transmissible, and the environment likely serves as a stable reservoir of infectious prions, facilitating a sustained incidence of CWD in free-ranging cervid populations and complicating efforts to eliminate disease in captive herds. Prions will enter the environment through mortalitie...
journal_title:Prion
pub_type: 杂志文章,评审
doi:10.4161/pri.2.4.7951
更新日期:2008-10-01 00:00:00
abstract::A case of L-type-like atypical bovine spongiform encephalopathy was detected in 14-year-old Japanese black beef cattle (BSE/JP24). To clarify the biological and biochemical properties of the prion in BSE/JP24, we performed a transmission study with wild-type mice and bovinized transgenic mice (TgBoPrP). The BSE/JP24 p...
journal_title:Prion
pub_type: 杂志文章
doi:10.4161/pri.2.3.7437
更新日期:2008-07-01 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 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::Prion protein (PrP), the causative agent of transmissible spongiform encephalopathies, is synthesized in the endoplasmic reticulum (ER) where it undergoes numerous covalent modifications. Here we investigate the interdependence and regulation of PrP oxidative folding, N-glycosylation and GPI addition in diverse ER con...
journal_title:Prion
pub_type: 杂志文章
doi:10.4161/pri.1.4.5727
更新日期:2007-10-01 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::Heat shock proteins HSP27, HSP70 and HSP90 are molecular chaperones whose expression is increased after many different types of stress. They have a protective function helping the cell to cope with lethal conditions. The cytoprotective function of HSPs is largely explained by their anti-apoptotic function. HSPs have b...
journal_title:Prion
pub_type: 杂志文章,评审
doi:10.4161/pri.1.1.4059
更新日期:2007-01-01 00:00:00