期刊
BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS
卷 365, 期 4, 页码 856-862出版社
ACADEMIC PRESS INC ELSEVIER SCIENCE
DOI: 10.1016/j.bbrc.2007.11.076
关键词
cystic fibrosis; CFTR; mitochondria; C10ORF70; mitoNEET; ZCD1; CISD1; CFTR inhibitors; mitochondrial failure; complex I
Cystic fibrosis (CF) is an autosomic recessive disease caused by mutations in the CFTR chloride channel, which indirectly affect the expression of a net of genes. Here we describe a new CFTR-dependent gene, CISD1, encoding for the first member of a family of proteins possessing a CDGSH signature. CISD1 mRNA is down-regulated in cystic fibrosis cells, and restored in the same cells ectopically expressing wt-CFTR (CFDE and CFDE/6RepCFTR; IB3-1 and S9 cells). Inhibition of CFTR chloride transport activity by using glibenclamide (50 mu M, 24 h) or CFTR(inh)-172 (5 mu M, 24 h), resulted in the down-regulation of CISD1 mRNA, and CFTR stimulation with cAMP/isoproterenol/IBMX upregulated its expression. As predicted by PSORT II, a CISD1-GFP chimera was found to be located into mitochondria, suggesting a possible role in the function/regulation of mitochondrial activity, in agreement with earlier observations of a possible mitochondrial failure in cystic fibrosis. (C) 2007 Elsevier Inc. All rights reserved.
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