Strain information
 NBRP Rat No: 0867  Strain name: FPL/Iet  Commmon Name: Fused pulmonary lobes, FPL Rat Genome Database
Principal Investigator:  Akira Sato  The Institute of Environmental Toxicology           303-0043      Japan
Tel: 0297-27-4537    Fax: 0297-27-4519 Email: a.satoh@iet.or.jp
Preservation Status:   Embryo        Sperm       Living Animals
Coat Color  アルビノ
Inbred Generations  F37
Usage Restrictions  In publishing, the research results to be obtained by the use of the BIOLOGICAL RESOURCE, a citation of the following literature(s) designated by the DEPOSITOR is requested.
Aoyama H, Teramoto S, Shirasu Y.
Heritable pulmonary lobation anomaly in the rat.
Teratol. 37:159-166, 1988.
Genetic Status
 Inbred  Segregating  Congenic  Consomic  Recombinant
 Coisogenic  Spont. Mutant  Transgene  Ind. Mutant  Category Other 
Comercial Availability
Research Category
 Diabetes Obesity  Neurobiology  Ophthalmology  Dentistry  Cardio Hypertension
 Cancer  Metabolism  Otorhinology  Immunology  Infectious
 Osteosis  Internal Organ  Dermatology  Reproduction  Development
 Behavior  Hematology  Urology  Pharmacology  Research Area Others 呼吸器疾患
 Control Strain  Marker Strain
Gene Affected ヒトにおけるFraser症候群の原因遺伝子の一つであり基底膜タンパク質をコードするFrem2のSer2005 が，CからAへの一塩基置換により終止コドンとなるナンセンス変異。
Origin This spontaneous mutant strain wa found in a colony of Jcl:Wistar strain at the Institute of Environmental Toxicology in 1978: 3 rats out of 13 rats showed fused right pulmonary lobes with reduction deformity of middle pulmonary lobes. These rats also had the syndactylism or paten tof the eyelid. It was difficult to maintain this strain in homozygous condition, mating system has been changed to sib mating between heterozygous rats.
Strain characteristics The hereditary pattern is autosomal recessive manner: nonsense mutation in Frem2 gene (one of the cousative gene of Fraser syndrome) was identified. In homozygous rats, fused right pulmonary lobes with reduction deformity of middle pulmonary lobes is obserbed, and complications with the syndactylism or patent of the eyelid are also seen. The mortality rate of homozygous rats during suckling period is 45-60% (in case of heterozygous mothers) or 90% (in case of homozygous mothers). The lungs of homozygous rat have congenital bronchial branching abnormalities. For detailed description about phenotypes of this strain, please check references.
Breeding Conditions Sib mating (between homozygous and heterozygous rats or between heterozygous rats).
Genotyping Allele specific PCR using Ampdirect® Plus（SHIMADZU). Primers are F: TCTACTTTGGGGATACCCAGCAGTC (for wild-type allele), F: TCTACTTTGGGGATACCCAGCAGTA (for mutant allele), and R: GAGAACCACACCGTGCAAAC. Product size is 345 bp. PCR condition: 94℃, 10 min, 30 cycles of 94℃, 30 sec, 55℃, 30 sec, and 72℃, 30 sec).
References *Aoyama H, Teramoto S, Shirasu Y.
Heritable pulmonary lobation anomaly in the rat.
Teratol. 37:159-166, 1988.

Aoyama H, Nakashima S, Teramoto S, Shirasu Y.
Congenital malformations in rats with the semilethal mutation fused pulmonary lobes.
Teratol. 41:113-122, 1990.

Aoyama H, Teramoto S, Shirasu Y.
Respiratory failure in newborn rats with semilethal mutation fused pulmonary lobes as a cause of neonatal death.
Cong Anom. 30:297-309, 1990.

Aoyama H, Fujii S, Hojo H, Teramoto S.
Bronchial branching abnormalities and emphysema-like changes in mutant rats having congenital lobation anomalies in the lung.
Cong Anom. 33:379-388, 1993.

Aoyama H, Fujii S, Hojo H, Teramoto S.
Pathogenetic changes in the lung bud of mutant rats with heritable pulmonary lobation anomalies.
Teratol. 50:117-183, 1994.

Kiyozumi D, Nakano I, Takahashi K, Hojo H, Aoyama H, Sekiguchi K.
Fused pulmonary lobes is a rat model of human Fraser syndrome.
Biochem Biophys Res Commun. 2011 Jul 29;411(2):440-444.
Additional strain information