B6;129P2-Cftr^tm1Unc/Orl

Status	Available to order
EMMA ID	EM:00051
International strain name	B6;129P2-Cftr^tm1Unc/Orl
Alternative name	Cftr°
Strain type	Targeted Mutant Strains : Knock-out
Allele/Transgene symbol	Cftr^tm1Unc
Gene/Transgene symbol	Cftr

Information from provider

Provider	Beverly H. Koller
Provider affiliation	c/o CDTA-Orleans
Phenotypic information	Mutant mice deficient in Cftr gene. Obtained by homologous recombination at exon 10 of the Cftr gene (insertion of the neomycin-resistance gene). No modification of the phenotype among the heterozygous population. Homozygotes show anomalies similar to human mucoviscidosis found in young patients. Most of these animals die of intestinal obstruction before mature age.
Breeding history	129/Ola ES cells; 3 generations of backcross to C57BL/6, then intercross. Breeding heterozygous x heterozygous.
References	An animal model for cystic fibrosis made by gene targeting.;Snouwaert J N, Brigman K K, Latour A M, Malouf N N, Boucher R C, Smithies O, Koller B H, ;1992;Science (New York, N.Y.);257;1083-8; 1380723

Information from EMMA

Archiving centre	Institut de Transgenose, INTRAGENE, Orléans, France
Animals used for archiving	heterozygous 129/Sv x C57BL/6, wild-type C57BL/6
Stage of embryos	2-cell

Disease and phenotype information

MGI allele-associated human disease models

cystic fibrosis / DOID:1485

Orphanet associated rare diseases, based on orthologous gene matching

Cystic fibrosis / Orphanet_586
Congenital bilateral absence of vas deferens / Orphanet_48

MGI phenotypes (allele matching)

impaired fertilization / MGI
reduced male fertility / MGI
decreased litter size / MGI
asthenozoospermia / MGI
impaired sperm capacitation / MGI
transmission ratio distortion / MGI
small ovary / MGI
abnormal uterine cervix morphology / MGI
decreased body weight / MGI
postnatal growth retardation / MGI
lung inflammation / MGI
reduced female fertility / MGI
delayed sexual maturation / MGI
small uterus / MGI
decreased corpora lutea number / MGI
intestinal obstruction / MGI
decreased ovulation rate / MGI
abnormal sperm physiology / MGI
abnormal phospholipid level / MGI
decreased ovary weight / MGI
decreased uterus weight / MGI
homeostasis/metabolism phenotype / MGI
prolonged estrous cycle / MGI
absent estrous cycle / MGI
thin uterus / MGI
pancreatic acinar cell zymogen granule accumulation / MGI
ileum hypertrophy / MGI
increased susceptibility to bacterial infection induced morbidity/mortality / MGI
postnatal lethality, incomplete penetrance / MGI
decreased respiratory epithelial chloride transmembrane transport / MGI
decreased respiratory epithelial sodium ion transmembrane transport / MGI
decreased intestinal epithelial sodium ion transmembrane transport / MGI
pancreatic acinus dilation / MGI
abnormal intestine morphology / MGI
abnormal bronchus morphology / MGI
abnormal pulmonary alveolus morphology / MGI
pulmonary interstitial fibrosis / MGI
increased lung compliance / MGI
abnormal crypts of Lieberkuhn morphology / MGI
abnormal colon morphology / MGI
spleen hyperplasia / MGI
decreased body size / MGI
distended abdomen / MGI
abnormal gait / MGI
abnormal thymus involution / MGI
female infertility / MGI
abnormal pancreas morphology / MGI
premature death / MGI
abnormal gland morphology / MGI
abnormal nasal mucosa morphology / MGI
abnormal paranasal sinus morphology / MGI
abnormal trachea morphology / MGI
peritoneal inflammation / MGI
abnormal submandibular gland morphology / MGI
small pancreas / MGI
abnormal gallbladder morphology / MGI
dilated gallbladder / MGI
coiled cecum / MGI
increased respiratory mucosa goblet cell number / MGI
abnormal Brunner's gland morphology / MGI
abnormal trachea gland morphology / MGI
abnormal intestinal goblet cell morphology / MGI
decreased intestinal epithelial chloride transmembrane transport / MGI
dilated crypts of Lieberkuhn / MGI
abnormal vital capacity / MGI
respiratory system phenotype / MGI

MGI phenotypes (gene matching)

decreased neutrophil cell number / MGI
impaired fertilization / MGI
abnormal intestine morphology / MGI
abnormal crypts of Lieberkuhn morphology / MGI
crypts of Lieberkuhn abscesses / MGI
abnormal colon morphology / MGI
abnormal small intestine morphology / MGI
abnormal intestinal mucosa morphology / MGI
intestinal ulcer / MGI
abnormal hepatocyte morphology / MGI
abnormal salivary gland morphology / MGI
spleen hyperplasia / MGI
small ovary / MGI
abnormal uterine cervix morphology / MGI
abnormal lung morphology / MGI
atelectasis / MGI
decreased body length / MGI
decreased body weight / MGI
weight loss / MGI
decreased body size / MGI
distended abdomen / MGI
abnormal lacrimal gland morphology / MGI
abnormal gait / MGI
aphagia / MGI
abnormal digestive system physiology / MGI
postnatal growth retardation / MGI
abnormal ion homeostasis / MGI
abnormal fluid regulation / MGI
abnormal thymus involution / MGI
lung inflammation / MGI
reduced male fertility / MGI
reduced female fertility / MGI
female infertility / MGI
decreased litter size / MGI
delayed sexual maturation / MGI
abnormal pancreas morphology / MGI
premature death / MGI
abnormal respiratory system physiology / MGI
abnormal gland morphology / MGI
no abnormal phenotype detected / MGI
abnormal nasal mucosa morphology / MGI
abnormal paranasal sinus morphology / MGI
abnormal bronchus morphology / MGI
abnormal pulmonary alveolus morphology / MGI
abnormal trachea morphology / MGI
abnormal vital capacity / MGI
increased susceptibility to infection / MGI
increased susceptibility to bacterial infection / MGI
small uterus / MGI
asthenozoospermia / MGI
decreased corpora lutea number / MGI
abnormal pancreas physiology / MGI
abnormal vas deferens morphology / MGI
abnormal bile duct morphology / MGI
no phenotypic analysis / MGI
gallbladder inflammation / MGI
intestinal obstruction / MGI
peritoneal inflammation / MGI
acute pancreas inflammation / MGI
decreased ovulation rate / MGI
pulmonary interstitial fibrosis / MGI
abnormal intestinal goblet cell morphology / MGI
impaired sperm capacitation / MGI
abnormal submandibular gland morphology / MGI
abnormal jejunum morphology / MGI
transmission ratio distortion / MGI
small pancreas / MGI
abnormal sperm physiology / MGI
abnormal phospholipid level / MGI
abnormal small intestine crypts of Lieberkuhn morphology / MGI
abnormal large intestine crypts of Lieberkuhn morphology / MGI
decreased ovary weight / MGI
decreased uterus weight / MGI
abnormal gallbladder morphology / MGI
abnormal gallbladder physiology / MGI
cachexia / MGI
liver/biliary system phenotype / MGI
homeostasis/metabolism phenotype / MGI
endocrine/exocrine gland phenotype / MGI
digestive/alimentary phenotype / MGI
craniofacial phenotype / MGI
respiratory system phenotype / MGI
reproductive system phenotype / MGI
abnormal alveolar macrophage morphology / MGI
decreased survivor rate / MGI
prolonged estrous cycle / MGI
absent estrous cycle / MGI
thin uterus / MGI
dilated pancreatic duct / MGI
pancreatic acinar cell atrophy / MGI
pancreatic acinar cell zymogen granule accumulation / MGI
enlarged gallbladder / MGI
dilated gallbladder / MGI
small cecum / MGI
coiled cecum / MGI
ileum hypertrophy / MGI
increased susceptibility to bacterial infection induced morbidity/mortality / MGI
abnormal intestine physiology / MGI
abnormal small intestinal crypt cell physiology / MGI
lethality, incomplete penetrance / MGI
increased respiratory mucosa goblet cell number / MGI
increased lung compliance / MGI
postnatal lethality, complete penetrance / MGI
postnatal lethality, incomplete penetrance / MGI
preweaning lethality, complete penetrance / MGI
abnormal Brunner's gland morphology / MGI
dilated Brunner's glands / MGI
lacrimal gland atrophy / MGI
abnormal trachea gland morphology / MGI
abnormal small intestine goblet cell morphology / MGI
abnormal colon goblet cell morphology / MGI
abnormal ileal goblet cell morphology / MGI
decreased respiratory epithelial chloride transmembrane transport / MGI
abnormal submucosal gland morphology / MGI
meconium ileus / MGI
decreased small intestinal villus number / MGI
decreased respiratory epithelial sodium ion transmembrane transport / MGI
increased respiratory epithelial sodium ion transmembrane transport / MGI
decreased intestinal epithelial sodium ion transmembrane transport / MGI
decreased intestinal epithelial chloride transmembrane transport / MGI
dilated crypts of Lieberkuhn / MGI
intestinal mucus accumulation / MGI
pancreatic acinus dilation / MGI
bile duct epithelium hyperplasia / MGI

Literature references

An animal model for cystic fibrosis made by gene targeting.;Snouwaert J N, Brigman K K, Latour A M, Malouf N N, Boucher R C, Smithies O, Koller B H, ;1992;Science (New York, N.Y.);257;1083-8; 1380723
Cdc42 is required for male germline niche development in mice.;Mori Yoshifumi, Takashima Seiji, Kanatsu-Shinohara Mito, Yi Zheng, Shinohara Takashi, ;2021;Cell reports;36;109550; 34407418

Information on how we integrate external resources can be found here

Order

Availabilities

Requesting frozen sperm or embryos is generally advisable wherever possible, in order to minimise the shipment of live mice.

Frozen embryos. Delivered in 4 weeks (after paperwork in place). €1740^*

Due to the dynamic nature of our processes strain availability may change at short notice. The local repository manager will advise you in these circumstances.

^* In addition users have to cover all the shipping costs (including the cost for returning dry-shippers, where applicable).

More details on pricing and delivery times

Practical information

Genotyping protocol

Example health report
(Current health report will be provided later)

Material Transfer Agreement (MTA)
For this strain no provider MTA is needed. Distribution is based on the EMMA conditions only.

EMMA conditions
Legally binding conditions for the transfer

Other EMMA strains

Not found what you were looking for? Search here for other strains available from EMMA.

B6;129P2-Cftrtm1Unc/Orl