原创力文档a non-brichos surfactant protein c mutation disrupts epithelial cell function and intercellular signalingnon-brichos表面活性剂蛋白质c突变破坏上皮细胞功能和细胞间的信号.pdfVIP
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a non-brichos surfactant protein c mutation disrupts epithelial cell function and intercellular signalingnon-brichos表面活性剂蛋白质c突变破坏上皮细胞功能和细胞间的信号
Woischnik et al. BMC Cell Biology 2010, 11:88
/1471-2121/11/88
RESEARCH ARTICLE Open Access
A non-BRICHOS surfactant protein c mutation
disrupts epithelial cell function and intercellular
signaling
1 1 1 1 1 1
Markus Woischnik , Christiane Sparr , Sunčana Kern , Tobias Thurm , Andreas Hector , Dominik Hartl ,
Gerhard Liebisch2 3 3 2 1*
, Surafel Mulugeta , Michael F Beers , Gerd Schmitz , Matthias Griese
Abstract
Background: Heterozygous mutations of SFTPC, the gene encoding surfactant protein C (SP-C), cause sporadic and
familial interstitial lung disease (ILD) in children and adults. The most frequent SFTPC mutation in ILD patients leads
to a threonine for isoleucine substitution at position 73 (I73T) of the SP-C preprotein (proSP-C), however little is
known about the cellular consequences of SP-CI73T expression.
Results: To address this, we stably expressed SP-CI73T in cultured MLE-12 alveolar epithelial cells. This resulted in
increased intracellular accumulation of proSP-C processing intermediates, which matched proSP-C species
recovered in bronchial lavage fluid from patients with this mutation. Exposure of SP-CI73T cells to drugs currently
used empirically in ILD therapy, cyclophosphamide, azathioprine, hydroxychloroquine or methylprednisolone,
enhanced expression of the chaperones HSP90, HSP70, calreticulin and calnexin. SP-CI73T mutants had decreased
intracellular phosphatidylcholine level (PC) and increased lyso-PC level without appreciable changes of other
phospholipids. Treatment with methylprednisolone or hydroxychloroquine partially restored these lipid alterations.
Furthermore, SP-CI73T
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