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Cftr-KO Mouse
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Cftr-KO Mouse
製品名
Cftr-KO Mouse
製品ID
C001890
系統名
C57BL/6JCya-Cftrem1/Cya
背景情報
C57BL/6JCya
状況
このマウス系統を論文で使用する場合は、「Cftr-KO Mouse(カタログ番号C001890)はサイアジェンから購入しました。」と引用してください。
Disease Animal Models
製品タイプ
年齢
遺伝子型
性別
数量
標準的な配送方法では、少なくとも3匹のヘテロ接合体キャリアを保証しています。ホモ接合体キャリアや指定された性別の個体の繁殖サービスも利用可能です。
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Disease Animal Models
基本情報
検証 Data
関連リソース
基本情報
遺伝子名
遺伝子別名
Abcc7
NCBI ID
染色体
Chr 6
MGI ID
さらに
系統詳細
Cystic Fibrosis (CF) is an autosomal recessive disorder causing severe damage to the lungs, digestive system, and other organs. It thickens mucus, sweat, and digestive fluids, blocking ducts and channels. The disease manifests as a persistent cough, hyperinflation of lung lobes, chronic nasal congestion, headaches, sleep disorders, digestive and reproductive system disorders, and nutritional and growth development disorders. CF is caused by mutations in the CF-transmembrane conductance regulator (CFTR) gene, which encodes a cAMP-dependent chloride ion channel protein. Abnormal CFTR function can cause transmembrane transport disorders of chloride ions and bicarbonate, leading to mucus obstruction in exocrine glands, and affecting respiration, digestion, endocrine, and reproduction [1-2].
Cftr-KO mice are a gene-knockout (KO) model. Using gene-editing technology, exons 5-6 of the Cftr gene in mice have been knocked out. This model can be used for research on the pathogenic mechanism of cystic fibrosis and the development of related treatment methods. Homozygous Cftr-KO mice start to die at 2 weeks of age, and prophylactic PEG treatment can improve their survival rate [3]. This strain requires feeding with intestinal cleansers to maintain survival after 3 weeks of age.
参考文献
Zeiher B G, Eichwald E, Smith J J, et al.A MouseModelfortheAF508Allele of Cystic Fibrosis[J].[2023-07-17].
Enrica F, Anna T, Tiziana J, et al. A Peptide Nucleic Acid against MicroRNA miR-145-5p Enhances the Expression of the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) in Calu-3 Cells[J]. Molecules, 2018, 23(1).
Sharma J, Abbott J, Klaskala L, Zhao G, Birket SE, Rowe SM. A Novel G542X CFTR Rat Model of Cystic Fibrosis Is Sensitive to Nonsense Mediated Decay. Front Physiol. 2020 Dec 16;11:611294.
系統作製戦略
The mouse Cftr gene contains 27 exons. The ATG start codon is located in exon 1, and the TAG stop codon is located in exon 27. In this strain, the regions of exons 5-6 were knocked out using gene-editing technology.

Figure 1. Gene editing strategy for Cftr-KO mice.
適用分野
Research on the pathogenesis and treatment methods of cystic fibrosis (CF).
検証 Data
1. Cftr knockout leads to enamel mineralization defects

Figure 2. Dental appearance of wild-type (WT) mice and heterozygous and homozygous Cftr-KO mice. In 3-4-week-old mice, the teeth of both WT and heterozygous Cftr-KO mice were a healthy light yellow color. In contrast, the teeth of homozygous Cftr-KO mice were white, which is a typical characteristic of the cystic fibrosis (CF) mouse model and results from abnormal enamel formation and mineralization caused by the loss of CFTR function.
2. Gene Expression

Figure 3. Gene expression detection in the large intestine, small intestine, and lung tissues of heterozygous and homozygous Cftr-KO mice and wild-type (WT) mice (3-week-old, n=3). RT-qPCR results showed that murine Cftr (mCftr) transcripts were detected in the large intestine, small intestine, and lung tissues of both heterozygous Cftr-KO mice and WT mice, while mCftr transcripts were not detected in multiple tissues of homozygous Cftr-KO mice. (Data are presented as mean ± SEM)
3. Survival Curve

Figure 4. Survival curve of homozygous Cftr-KO mice. Homozygous Cftr-KO mice began to die at 2 weeks of age, and preventive PEG treatment could improve their survival rate.
4. AB-PAS Staining Analysis
(1)Small intestine tissue

Figure 5. AB-PAS staining analysis of small intestine tissues from Cftr-KO mice (3-week-old, homozygous, n=3)*. The results showed that a small amount of mucus was observed in the intestinal lumen and between the intestinal villi of WT mice (red arrows). Compared with WT mice, homozygous Cftr-KO mice exhibited significant abnormal mucus phenotypes, including the accumulation of mucus substances in the intestinal glands (black arrows), an increase in the volume of goblet cells (green arrows), an increase in mucus in the intestinal lumen and between the intestinal villi (red arrows), and dilation of the intestinal glands (brown arrows).
*These results are representative images, and no significant gender differences were observed between groups. The samples were from mice that did not receive preventive PEG treatment. Scale bars: 500 µm for low-power magnification, 50 µm for high-power magnification.
(2)Tracheal tissue

Figure 6. AB-PAS staining analysis of tracheal tissues from Cftr-KO mice (3-week-old, homozygous, n=3). Compared with WT mice, the tracheal tissues of homozygous Cftr-KO mice showed obvious abnormal mucus phenotypes, including dilation of submucosal glands (red arrows) and an increase in mucus substances within the glands (black arrows).
*These results are representative images. No significant gender differences were observed between the groups. The samples were from mice that did not receive preventive PEG treatment. Scale bars: 500 µm for low-power magnification and 50 µm for high-power magnification.
(3)Lung tissue

Figure 7. AB-PAS Staining Analysis of Lung Tissues from Cftr-KO Mice (3 weeks old, homozygous, n=3). Results showed that mucus was observed in the bronchial lumen of only one sample in the wild-type (WT) group (indicated by green arrows), whereas mucus accumulation in the bronchial lumen was increased in most samples of the homozygous Cftr-KO group. Scale bar: 50 µm.
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