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Jens Christian Jensenius

The mannan-binding lectin pathway and lung disease in cystic fibrosis-disfunction of mannan-binding lectin-asssociated serine protease 2 (MASP-2) may be a major modifier

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The mannan-binding lectin pathway and lung disease in cystic fibrosis-disfunction of mannan-binding lectin-asssociated serine protease 2 (MASP-2) may be a major modifier. / Olesen, H.V.; Jensenius, Jens Christian; Steffensen, R.; Thiel, Steffen; Schiøtz, P.O.

In: Clin Immunol, Vol. 121, 2006, p. 324-31.

Research output: Contribution to journal/Conference contribution in journal/Contribution to newspaperJournal articleResearchpeer-review

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@article{809954b0c7d711dbbee902004c4f4f50,
title = "The mannan-binding lectin pathway and lung disease in cystic fibrosis-disfunction of mannan-binding lectin-asssociated serine protease 2 (MASP-2) may be a major modifier",
abstract = "The lectin pathway of complement activation is initiated by mannan-binding lectin (MBL) or the ficolins through the common MBL-associated serine protease-2 (MASP-2). Deficiency of MBL has been associated with poorer outcome in cystic fibrosis (CF). We investigated the MBL pathway further by analysis of the MASP-2 deficiency mutation (D105G) as well as MBL-2 genotypes. Concentrations and genotypes of MASP-2 and MBL in 109 CF patients were correlated to lung function and chronic infections. We describe the first CF patient homozygous for the mutation, a girl with extremely severe lung disease with no other precipitating factors. We suspect total MASP-2 dysfunction to be a major modifier of CF lung disease. However, heterozygosity for the D105G mutation of MASP-2 had no correlation to MBL pathway function or poor lung function. Lung function was higher in the MBL deficiency determining genotypes (XA/YO+YO/YO) than in the other genotypes",
author = "H.V. Olesen and Jensenius, {Jens Christian} and R. Steffensen and Steffen Thiel and P.O. Schi{\o}tz",
year = "2006",
language = "English",
volume = "121",
pages = "324--31",
journal = "Clin Immunol",

}

RIS

TY - JOUR

T1 - The mannan-binding lectin pathway and lung disease in cystic fibrosis-disfunction of mannan-binding lectin-asssociated serine protease 2 (MASP-2) may be a major modifier

AU - Olesen, H.V.

AU - Jensenius, Jens Christian

AU - Steffensen, R.

AU - Thiel, Steffen

AU - Schiøtz, P.O.

PY - 2006

Y1 - 2006

N2 - The lectin pathway of complement activation is initiated by mannan-binding lectin (MBL) or the ficolins through the common MBL-associated serine protease-2 (MASP-2). Deficiency of MBL has been associated with poorer outcome in cystic fibrosis (CF). We investigated the MBL pathway further by analysis of the MASP-2 deficiency mutation (D105G) as well as MBL-2 genotypes. Concentrations and genotypes of MASP-2 and MBL in 109 CF patients were correlated to lung function and chronic infections. We describe the first CF patient homozygous for the mutation, a girl with extremely severe lung disease with no other precipitating factors. We suspect total MASP-2 dysfunction to be a major modifier of CF lung disease. However, heterozygosity for the D105G mutation of MASP-2 had no correlation to MBL pathway function or poor lung function. Lung function was higher in the MBL deficiency determining genotypes (XA/YO+YO/YO) than in the other genotypes

AB - The lectin pathway of complement activation is initiated by mannan-binding lectin (MBL) or the ficolins through the common MBL-associated serine protease-2 (MASP-2). Deficiency of MBL has been associated with poorer outcome in cystic fibrosis (CF). We investigated the MBL pathway further by analysis of the MASP-2 deficiency mutation (D105G) as well as MBL-2 genotypes. Concentrations and genotypes of MASP-2 and MBL in 109 CF patients were correlated to lung function and chronic infections. We describe the first CF patient homozygous for the mutation, a girl with extremely severe lung disease with no other precipitating factors. We suspect total MASP-2 dysfunction to be a major modifier of CF lung disease. However, heterozygosity for the D105G mutation of MASP-2 had no correlation to MBL pathway function or poor lung function. Lung function was higher in the MBL deficiency determining genotypes (XA/YO+YO/YO) than in the other genotypes

M3 - Journal article

VL - 121

SP - 324

EP - 331

JO - Clin Immunol

JF - Clin Immunol

ER -