Cystic fibrosis transmembrane conductance regulator (CFTR)

Fertile (O'Neal et al., 1993)

No changes in lung, trachea, liver, or male reproductive tract (O'Neal et al., 1993)

Abnormalities of colon, lung, and vas deferens (Dorin et al., 1992)

Pathologic changes in intestines

Mucous accumulation in the crypts and intestinal lumen

Dilation of the bases of the crypts

Enlargment of goblet cells

Concretions in the crypts or between villi

Changes in the mucosal glands of the pharynx and minor sublingual glands

Dilation of acini

Accumulation of eosinophilic material

Changes in the pancreas

Atrophy of acinar cells

Mild inflammation of the main pancreatic duct

Defect in chloride ion transport

Absence of cAMP-activated chloride secretory response

Death (40%) within one week of birth from intestinal obstruction

Increased mRNA and protein expression of MUCILIN in pancreas and duodenum and not in gall bladder

Database link:

((cftr))

	Line Name	DNA Construct
1.	CFTR (+/-)	Lambda bacteriophages containing DNA using the CFTR (Cystic Fibrosis Transmembrane Regulator) gene of a strain 129 mouse were isolated, using a mouse CFTR cDNA probe, fr....
2.	CFTR (-/-)	Lambda bacteriophages containing DNA using the CFTR (Cystic Fibrosis Transmembrane Regulator) gene of a strain 129 mouse were isolated, using a mouse CFTR cDNA probe, fr....
3.	cftr (CAM) (+/-)	The targeting vector pCF-B(hprt)tk contains a 5.4 kb BamHI-KpnI fragment of mouse cftr sequence, surrounding the 180 bp exon 10. This was subclone....
4.	DELTA-F508 (+/-)	The targeting construct, p-DELTA-F508-1.9 was generated from pCF-B which contains a 5.4 kb genomic fragment spanning exon 10 of the Cftr gene. The DELTA-F508 mutation (....
5.	CFTR (+/-)	Lambda bacteriophages containing DNA using the CFTR (Cystic Fibrosis Transmembrane Regulator) gene of a strain 129 mouse were isolated, using a mouse CFTR cDNA probe, fr....
6.	CFTR (+/-)	The targeting vector CFneo(sup)2 (G00-345-113) was electroporated into E14TG2a cells. Targeted insertion of the neomycin gene inactivated the ES cells' cystic fibrosis t....
7.	cf/+	The insertional vector pIV3.5H was designed to disrupt the CFTR gene in exon 10. A 3.5 kb HindIII fragment of DNA isogenic with the E14 embryonic stem cell l....
8.	DELTA-F508 "Hit and	CFTR genomic DNA was cloned from a 129/Ola lambda library (provided by G. Grosveld; G00-100-384). A 2-kb EcoRI fragment containing exon 10 of the cftr gene w....
9.	CFTR-S489X	Lambda bacteriophages containing DNA using the CFTR (Cystic Fibrosis Transmembrane Regulator) gene of a strain 129 mouse were isolated, using a mouse CFTR cDNA probe, fr....
10.	CFTR-S489X	Lambda bacteriophages containing DNA using the CFTR (Cystic Fibrosis Transmembrane Regulator) gene of a strain 129 mouse were isolated, using a mouse CFTR cDNA probe, fr....
11.	CFTR-S489X	Lambda bacteriophages containing DNA using the CFTR (Cystic Fibrosis Transmembrane Regulator) gene of a strain 129 mouse were isolated, using a mouse CFTR cDNA probe, fr....
12.	CFTR (-/-)	Lambda bacteriophages containing DNA using the CFTR (Cystic Fibrosis Transmembrane Regulator) gene of a strain 129 mouse were isolated, using a mouse CFTR cDNA probe, fr....
13.	cftr (CAM) (-/-)	The targeting vector pCF-B(hprt)tk contains a 5.4 kb BamHI-KpnI fragment of mouse cftr sequence, surrounding the 180 bp exon 10. This was subclone....
14.	CFTR (-/-)	The targeting vector CFneo(sup)2 (G00-345-113) was electroporated into E14TG2a cells. Targeted insertion of the neomycin gene inactivated the ES cells' cystic fibrosis t....
15.	cf/cf	The insertional vector pIV3.5H was designed to disrupt the CFTR gene in exon 10. A 3.5 kb HindIII fragment of DNA isogenic with the E14 embryonic stem cell l....
16.	DELTA-F508 "Hit and	CFTR genomic DNA was cloned from a 129/Ola lambda library (provided by G. Grosveld; G00-100-384). A 2-kb EcoRI fragment containing exon 10 of the cftr gene w....
17.	DELTA-F508 (-/-)	The targeting construct, p-DELTA-F508-1.9 was generated from pCF-B which contains a 5.4 kb genomic fragment spanning exon 10 of the Cftr gene. The DELTA-F508 mutation (....