구독하기
연구 모델
서비스
전임상 효능 평가
Resource
Cftr-KO Mouse
제품 견적 요청
카탈로그에서 제품을 선택하여 요청을 제출해 주세요. Cyagen 팀이 상세 정보를 제공해 드립니다.
Cftr-KO Mouse
제품명
Cftr-KO Mouse
제품 ID
C001890
품종 계통
C57BL/6JCya-Cftrem1/Cya
Backgroud
C57BL/6JCya
상태
이 마우스 계통을 논문에서 사용할 경우, “Cftr-KO Mouse (카탈로그 번호 C001890)은 Cyagen에서 구입하였습니다.”라고 명시해 주시기 바랍니다.
Disease Animal Models
구매 가능한 제품 종류
연령
Genotype
성별
수량
표준 제공 조건은 최소 3마리의 이형접합(heterozygous) 보균자를 보장합니다. 동형접합(homozygous) 보균자 및/또는 특정 성별에 대한 브리딩 서비스도 제공됩니다.
가격 문의
Disease Animal Models
기본 정보
검증 데이터
관련 자료
기본 정보
유전자명
유전자 별칭
Abcc7
NCBI ID
염색체
Chr 6
MGI ID
Datasheet
품종 계통 설명
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.
Reference
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.
변형 전략
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.
응용 분야
Research on the pathogenesis and treatment methods of cystic fibrosis (CF).
검증 데이터
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.
문의하기
맞춤형 동물 모델 관련 상담을 위해 Cyagen 전문가와 연락해 보세요. 아래 양식을 작성하여 상담을 시작하거나 견적을 요청하시기 바랍니다.
Cyagen은 고객님의 개인정보를 소중히 여깁니다. 최신 제품, 서비스 및 인사이트를 안내드리고자 합니다. 고객님의 수신 설정은 다음과 같습니다:
해당 커뮤니케이션은 언제든지 수신 거부하실 수 있습니다. 수신 거부 방법 및 데이터 보호에 대한 자세한 내용은 개인정보처리방침을 참고해 주시기 바랍니다.
아래 버튼을 클릭함으로써, 요청하신 콘텐츠 제공을 위해 본 양식을 통해 제출된 개인정보를 Cyagen이 저장 및 처리하는 데 동의하게 됩니다.
