原發性肺纖維化病人基因表現與多型性之分析
Date Issued
2004-07-31
Date
2004-07-31
Author(s)
���R�R
DOI
922314B002304
Abstract
Idiopathic pulmonary fibrosis (IPF), is an interstitial lung disease of unknown cause,
characterized by the loss of alveolar architecture through the apoptosis of epithelial
and endothelial cells, proliferation of myofibroblasts , and extensive deposition of
extracellular matrix proteins, especially collagens type I and III. The molecular basis
of pulmonary fibrosis has been studied in a murine model. In mouse with pulmonary
fibrosis induced by bleomycin, the expression of a large cluster of genes were
augmented, and osteopontin was one of these most dramatically induced genes.
Osteopontin is an arginine-glycine-asparatic acid (RGD)-containing protein secreted
by a variety of cells, including osteoclasts, activated T cells, and activated
macrophages. There is evidence of alternative RNA splicing of the osteopontin gene
with three osteopontin cDNAs identified. The function of these splice variants is
unknown.
In the present study we examined the mRNA expression of osteopontin gene in
patients with idiopathic interstitial fibrosis IPF), acute interstitial pneumonitis (AIP)
and idiopathic pulmonary alveolar proteinosis (PAP), by RT-PCR and direct
sequencing of osteopontin mRNA transcripts. We found that the expression of a splice
variant mRNA of osteopontin gene, which lacks exon 5, is associated with severity
and poor prognosis of IPF, but not with prognosis of PAP. We are currently continuing
to collect more cases of idiopathic pulmonary fibrosis and other interstitial lung
disease which ends up with fibrosis, and examine the mRNA expression of
osteopontin gene, including its variant forms caused by alternative splicing.
Publisher
臺北市：國立臺灣大學醫學院檢驗醫學科
Type
journal article
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