Influence of the cystic fibrosis transmembrane conductance regulator on expression of lipid metabolism-related genes in dendritic cells.
Influence of the cystic fibrosis transmembrane conductance regulator on expression of lipid metabolism-related genes in dendritic cells.
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囊性纤维化跨膜电导调节剂对树突状细胞脂质代谢相关基因表达的影响。
DOI：
10.1186/1465-9921-10-26
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发表时间：
2009-04-03
影响因子：
5.8
通讯作者：
Worgall S
中科院分区：
文献类型：
--
作者：
Xu Y;Tertilt C;Krause A;Quadri LE;Crystal RG;Worgall S
Cystic fibrosis (CF) is caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene. Infections of the respiratory tract are a hallmark in CF. The host immune responses in CF are not adequate to eradicate pathogens, such as P. aeruginosa. Dendritic cells (DC) are crucial in initiation and regulation of immune responses. Changes in DC function could contribute to abnormal immune responses on multiple levels. The role of DC in CF lung disease remains unknown. This study investigated the expression of CFTR gene in bone marrow-derived DC. We compared the differentiation and maturation profile of DC from CF and wild type (WT) mice. We analyzed the gene expression levels in DC from naive CF and WT mice or following P. aeruginosa infection. CFTR is expressed in DC with lower level compared to lung tissue. DC from CF mice showed a delayed in the early phase of differentiation. Gene expression analysis in DC generated from naive CF and WT mice revealed decreased expression of Caveolin-1 (Cav1), a membrane lipid raft protein, in the CF DC compared to WT DC. Consistently, protein and activity levels of the sterol regulatory element binding protein (SREBP), a negative regulator of Cav1 expression, were increased in CF DC. Following exposure to P. aeruginosa, expression of 3β-hydroxysterol-Δ7 reductase (Dhcr7) and stearoyl-CoA desaturase 2 (Scd2), two enzymes involved in the lipid metabolism that are also regulated by SREBP, was less decreased in the CF DC compared to WT DC. These results suggest that CFTR dysfunction in DC affects factors involved in membrane structure and lipid-metabolism, which may contribute to the abnormal inflammatory and immune response characteristic of CF.
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影响因子：
2.4
作者：
Fernø J;Skrede S;Vik-Mo AO;Håvik B;Steen VM
通讯作者：
Steen VM
影响因子：
4.8
作者：
Kim, JH;Lee, JN;Paik, YK
通讯作者：
Paik, YK
DOI：
10.1073/pnas.1534923100
发表时间：
2003-10-14
影响因子：
11.1
作者：
Horton, JD;Shah, NA;Goldstein, JL
通讯作者：
Goldstein, JL
影响因子：
5.5
作者：
Assef, YA;Damiano, AE;Kotsias, BA
通讯作者：
Kotsias, BA
DOI：
10.1152/ajpgi.00035.2004
发表时间：
2004-08-01
影响因子：
4.5
作者：
Kaur, S;Norkina, O;De Lisle, RC
通讯作者：
De Lisle, RC