Abstract:
:The endoplasmic reticulum (ER) is an intracellular compartment dedicated to the synthesis and maturation of secretory and membrane proteins, totalling about 30% of the total eukaryotic cells proteome. The capacity to produce correctly folded polypeptides and to transport them to their correct intra- or extracellular destinations relies on proteostasis networks that regulate and balance the activity of protein folding, quality control, transport and degradation machineries. Nutrient and environmental changes, pathogen infection aging and, more relevant for the topics discussed in this review, mutations that impair attainment of the correct 3D structure of nascent polypeptide chains may compromise the activity of the proteostasis networks with devastating consequences on cells, organs and organisms' homeostasis. Here we present a review of mechanisms regulating folding and quality control of proteins expressed in the ER, and we describe the protein degradation and the ER stress pathways activated by the expression of misfolded proteins in the ER lumen. Finally, we highlight select examples of proteopathies (also known as conformational disorders or protein misfolding diseases) caused by protein misfolding in the ER and/or affecting cellular proteostasis and therapeutic interventions that might alleviate or cure the disease symptoms.
journal_name
Swiss Med Wklyjournal_title
Swiss medical weeklyauthors
Noack J,Brambilla Pisoni G,Molinari Mdoi
10.4414/smw.2014.14001subject
Has Abstractpub_date
2014-08-21 00:00:00pages
w14001eissn
1424-7860issn
1424-3997pii
smw-14001journal_volume
144pub_type
杂志文章,评审abstract::Understanding how best to manage the complex healthcare needs of hospitalised, mostly multimorbid medical patients is an international priority. Healthcare should be effective, safe and provide high quality at a reasonable cost. However, basic logistic and organisational issues of medical ward-based care have received...
journal_title:Swiss medical weekly
pub_type: 杂志文章
doi:10.4414/smw.2017.14515
更新日期:2017-10-22 00:00:00
abstract:STUDY OBJECTIVES:we performed an open-label national study to evaluate the effects of Bosentan on haemodynamics, exercise capacity, quality of life, safety and tolerability in patients with chronic thromboembolic pulmonary hypertension (CTEPH). PATIENTS AND METHODS:fifteen patients with CTEPH not eligible or waiting f...
journal_title:Swiss medical weekly
pub_type: 临床试验,杂志文章
doi:2007/41/smw-11819
更新日期:2007-10-20 00:00:00
abstract:QUESTION UNDER STUDY:To investigate the change over time in the number of ED admissions with positive blood alcohol concentration (BAC) and to evaluate predictors of BAC level. METHODS:We conducted a single site retrospective study at the ED of a tertiary referral hospital (western part of Switzerland) and obtained al...
journal_title:Swiss medical weekly
pub_type: 杂志文章
doi:10.4414/smw.2014.13982
更新日期:2014-08-07 00:00:00
abstract::Idiopathic pulmonary fibrosis (IPF) is a chronic and usually progressive lung disorder of unknown aetiology. Conventional management of patients with IPF has been primarily based on the concept that suppressing inflammation would prevent progression to fibrosis. Although the pathogenesis is incompletely understood, it...
journal_title:Swiss medical weekly
pub_type: 杂志文章,评审
doi:2003/29/smw-09986
更新日期:2003-07-26 00:00:00
abstract:BACKGROUND:In Switzerland, patients may undergo "blood tests" without being informed what these are screening for. Inadequate doctor-patient communication may result in patient misunderstanding. We examined what patients in the emergency department (ED) believed they had been screened for and explored their attitudes t...
journal_title:Swiss medical weekly
pub_type: 杂志文章
doi:10.4414/smw.2015.14206
更新日期:2015-12-04 00:00:00
abstract::The awareness of Cheyne-Stokes respiration (CSR) and of the co-existence of the obstructive sleep apnoea syndrome and central breathing disturbances has rapidly grown in recent years. CSR is defined by a waxing and waning pattern of the breathing amplitude. Sleep related breathing disorders in patients with heart fail...
journal_title:Swiss medical weekly
pub_type: 杂志文章,评审
doi:smw-12425
更新日期:2009-03-07 00:00:00
abstract:OBJECTIVE:To assess the clinical implementation and report preliminary results of a novel technique called the Ozaki procedure for stentless aortic valve replacement through reconstruction of the valve leaflets from autologous pericardium. METHOD:Between September 2015 and May 2017 30 patients (20 males, mean ± standa...
journal_title:Swiss medical weekly
pub_type: 杂志文章
doi:10.4414/smw.2018.14591
更新日期:2018-02-09 00:00:00
abstract::Each year, over 5 million people die worldwide from stroke, and at least every sixth patient who survives will experience another stroke within five years [1]. We are therefore eager to advance early and rapid diagnosis, prognosis and optimal risk stratification, as well as secondary prevention. In this context, blood...
journal_title:Swiss medical weekly
pub_type: 杂志文章,评审
doi:10.4414/smw.2015.14138
更新日期:2015-05-29 00:00:00
abstract::Better recipient selection, sophisticated postoperative surveillance and new immunosuppressive and anti-infective regimens can improve the results of lung transplantation. We compared the results of lung transplants performed between 1992 and 1996 (early period; 47) and between 1997 and 2000 (recent period; 46) in a c...
journal_title:Swiss medical weekly
pub_type: 杂志文章
doi:2001/17/smw-09718
更新日期:2001-05-05 00:00:00
abstract:BACKGROUND:Introduction of potent antiretroviral combination therapy (ART) has reduced overall morbidity and mortality amongst HIV-infected adults. Some prophylactic regimes against opportunistic infections can be discontinued in patients under successful ART. QUESTIONS UNDER STUDY:(1) The influence of the availabilit...
journal_title:Swiss medical weekly
pub_type: 杂志文章,多中心研究
doi:2001/31/smw-09728
更新日期:2001-08-11 00:00:00
abstract:BACKGROUND:since 1999 data from pulmonary hypertension (PH) patients from all PH centres in Switzerland were prospectively collected. We analyse the epidemiological aspects of these data. METHODS:PH was defined as a mean pulmonary artery pressure of >25 mm Hg at rest or >30 mm Hg during exercise. Patients with pulmona...
journal_title:Swiss medical weekly
pub_type: 杂志文章,多中心研究
doi:2008/25/smw-11915
更新日期:2008-06-28 00:00:00
abstract:BACKGROUND:Glomerulonephritis is a rare yet serious group of diseases with a high risk of progression to end-stage renal disease. For optimal healthcare planning, detailed epidemiological and demographic data are essential. Despite their clinical relevance, these data are largely lacking in Switzerland. OBJECTIVE:The ...
journal_title:Swiss medical weekly
pub_type: 杂志文章
doi:10.4414/smw.2020.20353
更新日期:2020-10-06 00:00:00
abstract:OBJECTIVES:Epidemological studies suggest that farmers are at an increased risk for chronic obstructive pulmonary disease because of exposure to organic dust. We assessed the proportion of farmers among patients with end stage lung disease and we measured the seasonal exposure of dairy farmers to organic dust in a rura...
journal_title:Swiss medical weekly
pub_type: 杂志文章
doi:2002/13/smw-09892
更新日期:2002-04-06 00:00:00
abstract:BACKGROUND:Several publications reported on the safety and efficacy of bortezomib, a novel first-in-class anti-cancer agent, in the treatment of relapsed/refractory multiple myeloma in controlled clinical trials. Complementary data on the experience with bortezomib in daily oncology practice are needed. We report on th...
journal_title:Swiss medical weekly
pub_type: 杂志文章
doi:2007/21/smw-11716
更新日期:2007-06-02 00:00:00
abstract:BACKGROUND:A clinical research application must be submitted for approval by a competent ethics committee (EC) before a study can be executed. There is very limited information on how such submissions could be optimised, especially regarding research in children and adolescents, which requires particular caution and ag...
journal_title:Swiss medical weekly
pub_type: 杂志文章
doi:10.4414/smw.2020.20267
更新日期:2020-06-19 00:00:00
abstract::Sports-practicing youths are at an elevated risk for alcohol use and misuse. Although much attention has recently been given to depicting subgroups facing the greatest threats, little evidence exists on the contexts in which their drinking takes place. Using data from a cross-sectional study on youth sports participat...
journal_title:Swiss medical weekly
pub_type: 杂志文章
doi:10.4414/smw.2012.13526
更新日期:2012-02-17 00:00:00
abstract:BACKGROUND:B-type natriuretic peptide (BNP) has been proven to be a biochemical marker of severity of congestive heart failure. We are aware of only few papers reporting the association of BNP elevation and pulmonary hypertension (primary, thromboembolic, or as a consequence of chronic obstructive pulmonary disease). L...
journal_title:Swiss medical weekly
pub_type: 杂志文章
doi:2003/37/smw-10367
更新日期:2003-09-26 00:00:00
abstract:QUESTIONS UNDER STUDY:To evaluate the diagnostic value of serum Cystatin C and urine neutrophil gelatinase-associated lipocalin (NGAL) for the detection of acute kidney injury in patients undergoing congenital heart surgery. METHODS:Serial samples of serum Cystatin C and urine NGAL were collected from 139 consecutive ...
journal_title:Swiss medical weekly
pub_type: 临床试验,杂志文章
doi:10.4414/smw.2013.13744
更新日期:2013-01-17 00:00:00
abstract:OBJECTIVE:The methods used to diagnose and classify ovarian cancer have changed over the past decade. We used hospital registry data to assess the incidence, treatment durations and hospital costs of ovarian cancer in Switzerland. METHODS:We carried out a retrospective analysis of a hospital registry covering all inpa...
journal_title:Swiss medical weekly
pub_type: 杂志文章
doi:10.4414/smw.2018.14647
更新日期:2018-07-29 00:00:00
abstract::We present the case of an oncology patient admitted to our hospital during the current COVID-19 pandemic with clinical and radiological features strongly suggestive of interstitial pneumonia. Multiple laboratory tests were negative for SARS-CoV-2 (polymerase chain reaction testing of nasopharyngeal swabs, and of induc...
journal_title:Swiss medical weekly
pub_type: 杂志文章
doi:10.4414/smw.2020.20312
更新日期:2020-07-11 00:00:00
abstract:BACKGROUND:To compare the efficacy of two different regimens in treatment of brucellosis. METHODS:This randomised clinical trial study was conducted on 280 patients with brucellosis in Babol, Iran, from April 1999 to January 2002. One of the following two regimens was randomly prescribed for two months: co-trimoxazole...
journal_title:Swiss medical weekly
pub_type: 临床试验,杂志文章,随机对照试验
doi:2004/37/smw-10665
更新日期:2004-09-18 00:00:00
abstract:INTRODUCTION:It has been proposed that reactive oxygen species play a role in renal fibrosis. 8-OHdG, a metabolite of oxidative damage to leukocyte DNA, has been identified as a marker of oxidative stress in patients with chronic renal failure. MATERIALS AND METHODS:Seventy-four patients following nephrectomy were ret...
journal_title:Swiss medical weekly
pub_type: 杂志文章
doi:10.4414/smw.2011.13268
更新日期:2011-10-12 00:00:00
abstract:OBJECTIVES:Several studies have reported prolonged QTc intervals in patients under methadone maintenance treatment, including development of torsade-de-pointes arrhythmia and death. It is still not clear why some patients develop critical QTc extensions while others do not. METHODS:ECG findings in a convenience sample...
journal_title:Swiss medical weekly
pub_type: 杂志文章
doi:10.4414/smw.2013.13852
更新日期:2013-09-06 00:00:00
abstract:OBJECTIVES:To investigate the subjective treatment goals of insulin-treated diabetic patients. METHODS:297 type 1 and 205 type 2 diabetic patients, representative of the North-western Swiss population, filled out a self report questionnaire focusing on their own treatment goals using standardized measures wherever ava...
journal_title:Swiss medical weekly
pub_type: 杂志文章
doi:2006/35/smw-11502
更新日期:2006-09-02 00:00:00
abstract::In Switzerland, voluntary termination of pregnancy (VTP) can be performed in all public and private hospitals with an obstetrics/gynaecology department. For various reasons, many Italian women use the Swiss healthcare system, in particular in Canton Ticino, a border region adjacent to Italy in the southern part of Swi...
journal_title:Swiss medical weekly
pub_type: 杂志文章
doi:10.4414/smw.2018.14636
更新日期:2018-07-09 00:00:00
abstract:BACKGROUND:In recent years, several treatment modalities have proved to be effective in the treatment of neuroendocrine tumours (NETs). However, there is currently no consensus on the sequence in which these options are best used. METHODS:In this observational study, we analysed the treatment modalities and sequences ...
journal_title:Swiss medical weekly
pub_type: 杂志文章
doi:10.4414/smw.2020.20176
更新日期:2020-01-15 00:00:00
abstract:QUESTIONS UNDER STUDY:Measuring the patient safety climate in the organisation of healthcare can help to identify problematic issues with a view to improving patient safety. We aimed (1) to describe the nurse-reported engagement in safety behaviours, (2) to describe the prevailing nurse-reported patient safety climate ...
journal_title:Swiss medical weekly
pub_type: 杂志文章,多中心研究
doi:10.4414/smw.2012.13501
更新日期:2012-01-20 00:00:00
abstract::Although progress has been made in the management of acute leukaemias, most patients who fail to respond to front-line therapies with cytostatic agents and stem cell transplantation, or who relapse after an initial response die from progressive disease. Novel treatment modalities exploiting donor-derived natural kille...
journal_title:Swiss medical weekly
pub_type: 杂志文章,评审
doi:
更新日期:2006-06-10 00:00:00
abstract:PRINCIPLES:Since the recommendation that infants sleep in the supine position, there has been an increase in cases of posterior positional plagiocephaly. Even though this condition is a purely cosmetic problem, if it is severe it may affect the child psychologically. Positioning may help in mild or moderate cases, but ...
journal_title:Swiss medical weekly
pub_type: 杂志文章
doi:2007/25/smw-11702
更新日期:2007-06-30 00:00:00
abstract:BACKGROUND:General practitioners (GPs) use diagnostic tests to help distinguish between viral and bacterial acute respiratory tract infections (ARTI). We investigated the prevalence of these tests, and how tests are associated with diagnosis, treatment and patient satisfaction. METHODS:As part of a clinical trial, 45 ...
journal_title:Swiss medical weekly
pub_type: 杂志文章,随机对照试验
doi:2006/15/smw-11343
更新日期:2006-04-15 00:00:00