Abstract:
:Cystic fibrosis (CF), is the most common life-shortening autosomal recessive disorder in Caucasians. It is caused by mutations in a single gene on the long arm of chromosome 7 that encodes the cystic fibrosis transmembrane conductance regulator (CFTR) protein. CF is characterized by abnormal Na+ and Cl- ion transport in several tissues, including the lungs, pancreas, gastrointestinal tract, liver, sweat glands, and male reproductive system. Progressive pulmonary disease is the dominant clinical feature of CF and accounts for morbidity and mortality. The inflammation characterized by an overabundance of activated neutrophils and macrophages on the respiratory epithelial surface is associated to a high production of reactive oxygen species (ROS) which contribute to the pathogenesis of cystic fibrosis. ROS could have different origins but the role of the NADPH oxidase system is essential. The "NADPH oxidases" (NOX/DUOX) family is an enzymatic complex formed by cytosolic and membrane subunits. Until now several homologues of the phagocytic NADPH oxidase have been identified in different tissues and it has been shown that the lungs preferentially expressed DUOX1-2. Thus, DUOX1-2 could be implicated in the anti-infectious defense system. The role of DUOX enzymes as a source of ROS in cystic fibrosis is examined as they could contribute to a better understanding of molecular mechanisms in CF. Moreover they could be a potential target for a new therapeutic approach.
journal_name
Ann Biol Clin (Paris)journal_title
Annales de biologie cliniqueauthors
Pongnimitprasert N,El-Benna J,Foglietti MJ,Gougerot-Pocidalo MA,Bernard M,Braut-Boucher Fdoi
10.1684/abc.2008.0285subject
Has Abstractpub_date
2008-11-01 00:00:00pages
621-9issue
6eissn
0003-3898issn
1950-6112pii
abc.2008.0285journal_volume
66pub_type
杂志文章,评审abstract::The introduction of the CRM 470 in 1993 (certified reference material for 14 serum proteins) and its utilization by industrial companies for cross-calibrating their commercial standards has been an important break-through in protein standardization. This improvement has been clearly illustrated by the last national qu...
journal_title:Annales de biologie clinique
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更新日期:1997-01-01 00:00:00
abstract::Using gas chromatography, we measured the concentrations of amino acids in a group of 80 selected subjects: we present the plasma values which we obtained, as well as those of other authors. Various comparisons were made, according to the age and/or sex of the subjects. We found that the plasma levels of valine, leuci...
journal_title:Annales de biologie clinique
pub_type: 杂志文章
doi:
更新日期:1983-01-01 00:00:00
abstract::Biermer's disease is an autoimmune atrophic gastritis of the fundus predominantly responsible for a malabsorption of vitamin B12. Despite its association with several autoimmune disorders, few observations have reported an association with autoimmune hemolytic anemia (AIHA). We report a case of Biermer's disease assoc...
journal_title:Annales de biologie clinique
pub_type: 杂志文章
doi:10.1684/abc.2012.0713
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journal_title:Annales de biologie clinique
pub_type: 杂志文章
doi:
更新日期:1991-01-01 00:00:00
abstract::In order to introduce the approach of HCV genotyping in our laboratory, a comparative study of 3 molecular and 1 serological methods, was conducted on 62 HCV RNA positive sera. The molecular genotyping methods target the 5'untranslated (UTR) region of the virus genome and are based on an amplification of the viral gen...
journal_title:Annales de biologie clinique
pub_type: 杂志文章
doi:
更新日期:2003-11-01 00:00:00
abstract::Monoclonal antibodies have been raised against porcine LH receptor and allowed to clone the corresponding messenger RNA from testicular cells. Cross hybridisation with the LH receptor clone allowed to isolate a clone corresponding to the human TSH receptor from thyroids. The structure of both receptors have been deter...
journal_title:Annales de biologie clinique
pub_type: 杂志文章,评审
doi:
更新日期:1992-01-01 00:00:00
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journal_title:Annales de biologie clinique
pub_type: 杂志文章
doi:10.1684/abc.2014.0979
更新日期:2014-09-01 00:00:00
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journal_title:Annales de biologie clinique
pub_type: 杂志文章
doi:
更新日期:1977-01-01 00:00:00
abstract::Until now, the methods of analysis by luminescence have not developed greatly in clinical biology because of the instability of the reagents and the absence of a single protocol applicable to a sufficiently wide range of assays. For this reason, the application has been limited to several assays using a specific biolu...
journal_title:Annales de biologie clinique
pub_type: 杂志文章,评审
doi:
更新日期:1985-01-01 00:00:00
abstract::Spontaneous red blood cell (RBC) fragmentation occurs in some membrane erythropathies like hereditary elliptocytosis (HE); this phenomenon is produced in normal RBC by heating at 49 degrees C, but not at temperatures below this limit; fragmentation is usually quantified by counting the number of fragments/1000 RBC und...
journal_title:Annales de biologie clinique
pub_type: 杂志文章
doi:
更新日期:1992-01-01 00:00:00
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journal_title:Annales de biologie clinique
pub_type: 杂志文章
doi:
更新日期:2006-11-01 00:00:00
abstract::Immunophenotyping is a major tool for the diagnosis of the chronic lymphoïd leukaemia (CLL). Its interest remains limited in the classification of the other B chronic lymphoproliférative syndromes (B-CLPS). We evaluate the place of the flow cytometry (CMF) in the diagnosis and classification of the non CLL B-CLPS. The...
journal_title:Annales de biologie clinique
pub_type: 杂志文章
doi:10.1684/abc.2013.0915
更新日期:2013-11-01 00:00:00
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journal_title:Annales de biologie clinique
pub_type: 杂志文章
doi:10.1684/abc.2015.1032
更新日期:2015-05-01 00:00:00
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journal_title:Annales de biologie clinique
pub_type: 杂志文章
doi:
更新日期:1990-01-01 00:00:00
abstract::Diagnosis of haematological malignancies is based on multiparametric analysis such as morphology, phenotype and genotype studies. Some entities are only defined by one of these approach. Flow-cytometry (FCM) is useful to determined the normal counterpart of the tumoral process and its differentiation status within the...
journal_title:Annales de biologie clinique
pub_type: 杂志文章,评审
doi:
更新日期:2002-11-01 00:00:00
abstract::Nowadays, physiological amino acids profiling is based primarily on ion exchange chromatography (IEC) coupled to a post-column derivatization with ninhydrin and UV detection at two wavelengths. Unfortunately, this technique suffers various drawbacks such as long analysis time, high sample volume and specific costs rel...
journal_title:Annales de biologie clinique
pub_type: 杂志文章
doi:10.1684/abc.2015.1066
更新日期:2015-07-01 00:00:00
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journal_title:Annales de biologie clinique
pub_type: 杂志文章
doi:10.1684/abc.2017.1225
更新日期:2017-04-01 00:00:00
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journal_title:Annales de biologie clinique
pub_type: 杂志文章
doi:
更新日期:1984-01-01 00:00:00
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journal_title:Annales de biologie clinique
pub_type: 杂志文章,评审
doi:
更新日期:1998-09-01 00:00:00
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journal_title:Annales de biologie clinique
pub_type: 杂志文章
doi:
更新日期:1977-01-01 00:00:00
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journal_title:Annales de biologie clinique
pub_type: 杂志文章
doi:
更新日期:1986-01-01 00:00:00
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journal_title:Annales de biologie clinique
pub_type: 杂志文章
doi:10.1684/abc.2008.0274
更新日期:2008-09-01 00:00:00
abstract::Cystic fibrosis (CF) is the most common severe recessive disease in Caucasians. The gene responsible for the disease encodes a protein named cystic fibrosis transmembrane conductance regulator, which is predicted to function as a cAMP-regulated chloride channel. Reduced chloride secretion is at the basis of insufficie...
journal_title:Annales de biologie clinique
pub_type: 杂志文章,评审
doi:
更新日期:1996-01-01 00:00:00
abstract::Several biological parameters for assessing selenium status have been determined in years 1985-1986 in a large Belgian population group, males and females 0 to 92 years old, representative from Brussels and surroundings. In 145 people, 20 to 79 years old, mean concentrations were: 1.06 +/- 0.15 mumol Se/l plasma, 5.0 ...
journal_title:Annales de biologie clinique
pub_type: 杂志文章
doi:
更新日期:1989-01-01 00:00:00
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journal_title:Annales de biologie clinique
pub_type: 杂志文章
doi:
更新日期:1996-01-01 00:00:00
abstract::Acute leukemia of megakaryocyte lineage (AML-7) is a rare entity defined by a blastic proliferation of which a part (>or= 50%) is represented by megakaryoblasts. We report the case of a 95 year old woman presenting a AML-7 secondary to a myelodysplastic syndrome (MDS), that represents an unusual form of MDS acutisatio...
journal_title:Annales de biologie clinique
pub_type: 杂志文章
doi:
更新日期:2006-03-01 00:00:00
abstract::A 58-year-old patient, without any notable medical history, except for alcoholism and treated hypertension, developed anemia and leukopenia with macrocytosis. Folate deficiency was diagnosed and subsequently treated. Despite folate supplementation, the hematological parameters did not normalize. Further diagnosis inve...
journal_title:Annales de biologie clinique
pub_type: 杂志文章
doi:10.1684/abc.2013.0863
更新日期:2013-07-01 00:00:00
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journal_title:Annales de biologie clinique
pub_type: 杂志文章
doi:10.1684/abc.2011.0533
更新日期:2011-03-01 00:00:00
abstract::The measurement of CDT (Carbohydrate Deficient Transferrin) is an essential biological tool in the diagnosis and follow-up of alcohol abuse. It is also employed as a marker of abstinence for the restitution of driving licences. However, the precision of measurement, and the between laboratory homogeneity of the result...
journal_title:Annales de biologie clinique
pub_type: 杂志文章,多中心研究
doi:
更新日期:2007-03-01 00:00:00
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journal_title:Annales de biologie clinique
pub_type: 杂志文章
doi:
更新日期:1993-01-01 00:00:00