RGD Reference Report - Elevated BALF concentrations of alpha- and beta-defensins in patients with pulmonary alveolar proteinosis. - Rat Genome Database

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Elevated BALF concentrations of alpha- and beta-defensins in patients with pulmonary alveolar proteinosis.

Authors: Mukae, H  Ishimoto, H  Yanagi, S  Ishii, H  Nakayama, S  Ashitani, J  Nakazato, M  Kohno, S 
Citation: Mukae H, etal., Respir Med. 2007 Apr;101(4):715-21. Epub 2006 Sep 26.
RGD ID: 4892262
Pubmed: PMID:17000097   (View Abstract at PubMed)
DOI: DOI:10.1016/j.rmed.2006.08.018   (Journal Full-text)

Defensins are endogenous antibiotics and regulators of inflammation, immunity and wound repair. Their concentrations are substantially increased in bronchoalveolar lavage fluid (BALF) of patients with infectious lung diseases. alpha-defensin (HAD) levels are also elevated in patients with idiopathic pulmonary fibrosis (IPF) and correlated with the decline in pulmonary function tests, suggesting the association of defensins with the pathogenesis of interstitial lung diseases. The aim of this study was to determine the profile of defensins in interstitial lung diseases. Serum and BALF levels of HAD and beta-defensin 1 and 2 (HBD-1, and -2) were measured by radioimmunoassay in 63 patients with interstitial lung diseases, including idiopathic pulmonary alveolar proteinosis (PAP), IPF, nonspecific interstitial pneumonia (NSIP), cryptogenic organizing pneumonia (COP) and pulmonary sarcoidosis, and in 9 healthy volunteers as controls. Levels of HAD in BALF of patients with PAP were significantly higher than those in controls and patients with COP and sarcoidosis. Serum levels of HAD in all groups were significantly higher than those in controls. Levels of HBD-1 and -2 in BALF of patients with PAP were extremely high in all subjects. Serum levels of HBD-1 were higher in all patient groups, with the exception of those with PAP, and those of HBD-2 were also higher in patients with IPF and sarcoidosis, compared with controls. BALF of PAP patients, but not IPF patients and controls, expressed antimicrobial activity against Pseudomonas aeruginosa and Staphylococcus aureus. Our findings suggest different kinetics of HAD and HBD-1 and -2 in serum and BALF of interstitial lung diseases and that these antimicrobial peptides in the airway lumen may contribute to prevention of bacterial airway infections in PAP.



RGD Manual Disease Annotations    Click to see Annotation Detail View

  
Object SymbolSpeciesTermQualifierEvidenceWithNotesSourceOriginal Reference(s)
DEFB1Humanpneumonia  IEP protein:increased expression:serumRGD 
Defb1Mousepneumonia  ISODEFB1 (Homo sapiens)protein:increased expression:serumRGD 
Defb1Ratpneumonia  ISODEFB1 (Homo sapiens)protein:increased expression:serumRGD 
DEFB1Humanpulmonary alveolar proteinosis  IEP protein:increased expression:respiratory system fluid/secretionRGD 
DEFB4AHumanpulmonary alveolar proteinosis  IEP protein:increased expression:respiratory system fluid/secretionRGD 
Defb1Ratpulmonary alveolar proteinosis  ISODEFB1 (Homo sapiens)protein:increased expression:respiratory system fluid/secretionRGD 
Defb1Mousepulmonary alveolar proteinosis  ISODEFB1 (Homo sapiens)protein:increased expression:respiratory system fluid/secretionRGD 
Defb4Ratpulmonary alveolar proteinosis  ISODEFB4A (Homo sapiens)protein:increased expression:respiratory system fluid/secretionRGD 
Defb4Mousepulmonary alveolar proteinosis  ISODEFB4A (Homo sapiens)protein:increased expression:respiratory system fluid/secretionRGD 
DEFB1Humanpulmonary fibrosis  IEP protein:increased expression:serumRGD 
DEFB4AHumanpulmonary fibrosis  IEP protein:increased expression:serumRGD 
Defb1Mousepulmonary fibrosis  ISODEFB1 (Homo sapiens)protein:increased expression:serumRGD 
Defb1Ratpulmonary fibrosis  ISODEFB1 (Homo sapiens)protein:increased expression:serumRGD 
Defb4Ratpulmonary fibrosis  ISODEFB4A (Homo sapiens)protein:increased expression:serumRGD 
Defb4Mousepulmonary fibrosis  ISODEFB4A (Homo sapiens)protein:increased expression:serumRGD 
DEFB1Humanpulmonary sarcoidosis  IEP protein:increased expression:serumRGD 
DEFB4AHumanpulmonary sarcoidosis  IEP protein:increased expression:serumRGD 
Defb1Mousepulmonary sarcoidosis  ISODEFB1 (Homo sapiens)protein:increased expression:serumRGD 
Defb1Ratpulmonary sarcoidosis  ISODEFB1 (Homo sapiens)protein:increased expression:serumRGD 
Defb4Ratpulmonary sarcoidosis  ISODEFB4A (Homo sapiens)protein:increased expression:serumRGD 
Defb4Mousepulmonary sarcoidosis  ISODEFB4A (Homo sapiens)protein:increased expression:serumRGD 

Objects Annotated

Genes (Rattus norvegicus)
Defb1  (defensin beta 1)
Defb4  (defensin beta 4)

Genes (Mus musculus)
Defb1  (defensin beta 1)
Defb4  (defensin beta 4)

Genes (Homo sapiens)
DEFB1  (defensin beta 1)
DEFB4A  (defensin beta 4A)


Additional Information