Abstract:
:Creutzfeldt-Jakob disease (CJD) and other transmissible spongiform encephalopathies (TSEs) are characterised by the accumulation of a pathological conformer of PrP, named PrPsc. Molecular weight and glycosylation of the protease-resistant core of PrPsc (PrP27-30) are heterogeneous in different forms of TSEs. We analysed PrP27-30 glycotypes in a large number of TSE-affected patients: 50 sporadic CJD (sCJD), 1 iatrogenic CJD, 1 Gerstmann-Sträussler-Scheinker syndrome (GSS) with the Pro102Leu mutation of PrP, 3 familial CJD (fCJD) with the Glu200Lys mutation and, for the first time, 7 fCJD with the Val210ll3e mutation. All patients were screened for the polymorphic codon 129 of the PrP gene. PrP27-30 deglycosylation and PrPsc immunohistochemistry were performed in selected cases. We found that two PrP27-30 glycotypes (type 1A and type 2A) are produced in sCJD. Type 1A is more frequently associated with methionine than valine in position 129. Type 1A is also formed in Val210lle fCJD. In Glu200Lys fCJD and GSS patients, we found that PrP27-30 has the same mobility of type 1 but different glycosylation ratios (type 1B). Our findings indicate that the polymorphic residue 129 of PrP has a leading role in determining the proteinase degradation site of PrPsc while mutant residues 102 or 200 influence only the glycosylation pattern.
journal_name
Brain Res Bulljournal_title
Brain research bulletinauthors
Cardone F,Liu QG,Petraroli R,Ladogana A,D'Alessandro M,Arpino C,Di Bari M,Macchi G,Pocchiari Mdoi
10.1016/s0361-9230(99)00077-5keywords:
subject
Has Abstractpub_date
1999-08-01 00:00:00pages
429-33issue
6eissn
0361-9230issn
1873-2747pii
S0361-9230(99)00077-5journal_volume
49pub_type
杂志文章abstract::The uptake of 3H-2-deoxyglucose (3H-2-DG) in selected brain structures was determined subsequent to electrical stimulation of insular cortex (Ins) and the mediodorsal (MD) nucleus of the thalamus in rabbits. Stimulation of Ins elicited parasympathetic-like responding (i.e., bradycardia and depressor responses); wherea...
journal_title:Brain research bulletin
pub_type: 杂志文章
doi:10.1016/0361-9230(87)90148-1
更新日期:1987-10-01 00:00:00
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journal_title:Brain research bulletin
pub_type: 杂志文章
doi:10.1016/0361-9230(94)90252-6
更新日期:1994-01-01 00:00:00
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journal_title:Brain research bulletin
pub_type: 杂志文章
doi:10.1016/0361-9230(93)90305-u
更新日期:1993-01-01 00:00:00
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journal_title:Brain research bulletin
pub_type: 杂志文章
doi:10.1016/0361-9230(89)90058-0
更新日期:1989-02-01 00:00:00
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journal_title:Brain research bulletin
pub_type: 杂志文章
doi:10.1016/0361-9230(90)90094-g
更新日期:1990-03-01 00:00:00
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journal_title:Brain research bulletin
pub_type: 杂志文章
doi:10.1016/j.brainresbull.2009.03.003
更新日期:2009-06-30 00:00:00
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journal_title:Brain research bulletin
pub_type: 杂志文章
doi:10.1016/j.brainresbull.2004.11.014
更新日期:2005-04-15 00:00:00
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journal_title:Brain research bulletin
pub_type: 杂志文章
doi:10.1016/0361-9230(86)90139-5
更新日期:1986-05-01 00:00:00
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journal_title:Brain research bulletin
pub_type: 杂志文章
doi:10.1016/j.brainresbull.2008.04.002
更新日期:2008-08-15 00:00:00
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journal_title:Brain research bulletin
pub_type: 杂志文章
doi:10.1016/0361-9230(82)90072-7
更新日期:1982-04-01 00:00:00
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journal_title:Brain research bulletin
pub_type: 杂志文章
doi:10.1016/0361-9230(93)90215-w
更新日期:1993-01-01 00:00:00
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journal_title:Brain research bulletin
pub_type: 杂志文章
doi:10.1016/s0361-9230(80)80013-x
更新日期:1980-07-01 00:00:00
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journal_title:Brain research bulletin
pub_type: 杂志文章
doi:10.1016/j.brainresbull.2006.06.006
更新日期:2006-10-16 00:00:00
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journal_title:Brain research bulletin
pub_type: 杂志文章
doi:10.1016/j.brainresbull.2004.07.006
更新日期:2004-09-30 00:00:00
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journal_title:Brain research bulletin
pub_type: 杂志文章
doi:10.1016/j.brainresbull.2009.05.005
更新日期:2009-08-14 00:00:00
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journal_title:Brain research bulletin
pub_type: 杂志文章
doi:10.1016/0361-9230(89)90193-7
更新日期:1989-12-01 00:00:00
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journal_title:Brain research bulletin
pub_type: 杂志文章
doi:10.1016/0361-9230(92)90044-x
更新日期:1992-03-01 00:00:00
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journal_title:Brain research bulletin
pub_type: 杂志文章
doi:10.1016/s0361-9230(00)00337-3
更新日期:2000-09-15 00:00:00
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journal_title:Brain research bulletin
pub_type: 杂志文章
doi:10.1016/0361-9230(87)90056-6
更新日期:1987-12-01 00:00:00
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journal_title:Brain research bulletin
pub_type: 杂志文章
doi:10.1016/0361-9230(83)90083-7
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journal_title:Brain research bulletin
pub_type: 杂志文章
doi:10.1016/j.brainresbull.2007.11.016
更新日期:2008-03-28 00:00:00
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journal_title:Brain research bulletin
pub_type: 杂志文章
doi:10.1016/j.brainresbull.2020.01.017
更新日期:2020-04-01 00:00:00
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journal_title:Brain research bulletin
pub_type: 杂志文章
doi:10.1016/0361-9230(93)90191-d
更新日期:1993-01-01 00:00:00
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journal_title:Brain research bulletin
pub_type: 杂志文章
doi:10.1016/0361-9230(82)90034-x
更新日期:1982-01-01 00:00:00
abstract::Thyroid-stimulating hormone (TSH) stimulates the synthesis and release of thyroid hormones including triiodothyronine (T3) and thyroxine (T4). Semiquantitative analyses using northern blot and in situ hybridization suggested that TSH gene transcription is upregulated under conditions of hypothyroidism. However, no qua...
journal_title:Brain research bulletin
pub_type: 杂志文章
doi:10.1016/j.brainresbull.2007.06.004
更新日期:2007-09-14 00:00:00
abstract::This paper surveys more than twenty types of tactile illusions and discusses several of their aspects. These aspects include the ease with which they can be demonstrated and whether they have clear visual analogs. The paper also shows how to construct equipment made of simple supplies able to deliver well-controlled t...
journal_title:Brain research bulletin
pub_type: 杂志文章,评审
doi:10.1016/j.brainresbull.2008.01.008
更新日期:2008-04-15 00:00:00
abstract::The effect of the intracerebroventricular (ICV) administration of pertussis toxin on penile erection and yawning induced by apomorphine and oxytocin was studied in male rats. Pertussis toxin (2 micrograms ICV) prevented the above behavioral responses to apomorphine (80 micrograms/kg SC) and oxytocin (30 ng ICV) on day...
journal_title:Brain research bulletin
pub_type: 杂志文章
doi:10.1016/0361-9230(92)90194-3
更新日期:1992-02-01 00:00:00
abstract::Previously, we have showed that the cholecystokinin (CCK)-A receptor expression in hypothalamus is closely related with the responsiveness of electroacupuncture (EA)-mediated analgesic effects in rats. In order to confirm this observation more directly in vivo, the EA-mediated analgesic effects are compared between Ot...
journal_title:Brain research bulletin
pub_type: 杂志文章
doi:10.1016/j.brainresbull.2003.09.007
更新日期:2003-12-15 00:00:00
abstract::The analysis of physiological processes in brain by position emission tomography (PET) is facilitated when images are spatially normalized to a standard coordinate system. Thus, PET activation studies of human brain frequently employ the common stereotaxic coordinates of Talairach. We have developed an analogous stere...
journal_title:Brain research bulletin
pub_type: 杂志文章
doi:10.1016/j.brainresbull.2005.02.033
更新日期:2005-07-15 00:00:00
abstract::Many objective and quantitative methods have been developed to create a procedure or a device to prove, describe and quantify olfactory deficit and anosmia, especially after a head trauma. Electrophysiological testing throughout olfactoelectroencephalography (olfactoEEG) is based on brain activity desynchronisation, a...
journal_title:Brain research bulletin
pub_type: 杂志文章
doi:10.1016/j.brainresbull.2006.08.004
更新日期:2006-12-11 00:00:00