Catalogo Articoli (Spogli Riviste)

OPAC HELP

Titolo:
The mannose binding lectin gene influences the severity of chronic liver disease in cystic fibrosis
Autore:
Gabolde, M; Hubert, D; Guilloud-Bataille, M; Lenaerts, C; Feingold, J; Besmond, C;
Indirizzi:
Hop Robert Debre, INSERM, U458, F-75019 Paris, France Hop Robert Debre Paris France F-75019 NSERM, U458, F-75019 Paris, France Hop Cochin, Serv Pneumophtisol, F-75979 Paris 14, France Hop Cochin Paris France 14 Serv Pneumophtisol, F-75979 Paris 14, France Hop Nord, Serv Pediat, F-80054 Amiens 1, France Hop Nord Amiens France 1Hop Nord, Serv Pediat, F-80054 Amiens 1, France Hop Necker Enfants Malad, INSERM, U393, F-75743 Paris 15, France Hop Necker Enfants Malad Paris France 15 U393, F-75743 Paris 15, France
Titolo Testata:
JOURNAL OF MEDICAL GENETICS
fascicolo: 5, volume: 38, anno: 2001,
pagine: 310 - 311
SICI:
0022-2593(200105)38:5<310:TMBLGI>2.0.ZU;2-P
Fonte:
ISI
Lingua:
ENG
Soggetto:
ASSOCIATION; INFECTION; MUTATIONS; CHILDREN;
Keywords:
cystic fibrosis; cirrhosis; mannose binding lectin; modulating gene;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Clinical Medicine
Life Sciences
Citazioni:
13
Recensione:
Indirizzi per estratti:
Indirizzo: Besmond, C Hop Robert Debre, INSERM, U458, 48 Blvd Serurier, F-75019 Paris, France Hop Robert Debre 48 Blvd Serurier Paris France F-75019 France
Citazione:
M. Gabolde et al., "The mannose binding lectin gene influences the severity of chronic liver disease in cystic fibrosis", J MED GENET, 38(5), 2001, pp. 310-311

Abstract

Chronic liver disease is a major complication of cystic fibrosis. Its incidence and severity show marked heterogeneity, even among the homogeneous group of homozygous Delta F508 patients, suggesting that environmental or genetic factors other than the deletion Delta F508 may influence the development of cystic fibrosis related liver disease. We investigated whether the allelic variants of mannose binding lectin, an important protein of the immune system, could be associated with the presence of cirrhosis in a population of 216 homogeneous homozygous Delta F508 patients. Analysis of the data shows that the presence of cirrhosis in cystic fibrosis patients is significantly associated with a mutated mannose binding lectin genotype (homozygousor compound heterozygous for mannose binding lectin variants). The modulating role of mannose binding lectin in the occurrence of cirrhosis in cysticfibrosis could be explained by the fact that hepatotoxic damage from viruses or bacteria might be increased by the immunodeficiency associated with mannose binding lectin variants and might facilitate the degradation of liver status. These data highlight the crucial role of mannose binding lectin in the clinical outcome of cystic fibrosis, as it has recently been shown that the mannose binding lectin gene is a modulating gene of the respiratory involvement in cystic fibrosis patients.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 31/03/20 alle ore 09:51:33