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01000naa a22002652 4500 |
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20231223105943.0 |
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231223s2006 xx ||||| 00| ||eng c |
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|a pubmed24n0554.xml
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|a (NLM)17045845
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|a DE-627
|b ger
|c DE-627
|e rakwb
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|a eng
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| 100 |
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|a Olesen, H V
|e verfasserin
|4 aut
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| 245 |
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|a The mannan-binding lectin pathway and lung disease in cystic fibrosis--disfunction of mannan-binding lectin-associated serine protease 2 (MASP-2) may be a major modifier
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|c 2006
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|a Text
|b txt
|2 rdacontent
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|a ohne Hilfsmittel zu benutzen
|b n
|2 rdamedia
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|a Band
|b nc
|2 rdacarrier
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|a Date Completed 05.01.2007
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|a Date Revised 20.11.2006
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|a published: Print-Electronic
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|a Citation Status MEDLINE
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|a 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
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|a Journal Article
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|a Research Support, Non-U.S. Gov't
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| 650 |
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|a Mannose-Binding Lectin
|2 NLM
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| 650 |
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|a MASP2 protein, human
|2 NLM
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| 650 |
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|a EC 3.4.21.-
|2 NLM
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| 650 |
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|a Mannose-Binding Protein-Associated Serine Proteases
|2 NLM
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| 650 |
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|a EC 3.4.21.-
|2 NLM
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| 700 |
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|a Jensenius, J C
|e verfasserin
|4 aut
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| 700 |
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|a Steffensen, R
|e verfasserin
|4 aut
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| 700 |
1 |
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|a Thiel, S
|e verfasserin
|4 aut
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| 700 |
1 |
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|a Schiøtz, P O
|e verfasserin
|4 aut
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| 773 |
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|i Enthalten in
|t Clinical immunology (Orlando, Fla.)
|d 1999
|g 121(2006), 3 vom: 01. Dez., Seite 324-31
|w (DE-627)NLM098196855
|x 1521-7035
|7 nnns
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| 773 |
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|g volume:121
|g year:2006
|g number:3
|g day:01
|g month:12
|g pages:324-31
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|a AR
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|d 121
|j 2006
|e 3
|b 01
|c 12
|h 324-31
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