Description

The genotypes of the animals provided may not reflect those discussed in the strain description or the mating scheme utilized by The Jackson Laboratory prior to cryopreservation. Please inquire for possible genotypes for this specific strain.

Strain Information

Former Names B6 x CBy.AK-Tgcog/J    (Changed: 25-JUL-07 ) B6 x CBy.AK-Tgncog/J    (Changed: 19-OCT-06 ) B6 x C.AK-Tgncog/+    (Changed: 15-DEC-04 ) Type Congenic; Mutant Strain; Spontaneous Mutation; Additional information on Genetically Engineered and Mutant Mice. Visit our online Nomenclature tutorial. Additional information on Congenic nomenclature. Species laboratory mouse Generation N1p

Description
The Tgn^cog mutation causes the development of goiters due to failed processing of thyroglobulin. Homozygotes are smaller in overall size by 15 days of age. They have an increase in growth rate at the time of weaning but generally do not attain comparable size with their wildtype littermates. Increased thyroidal volume is apparent at embryonic day 18 and continues enlarging to an average of 5 fold higher than normal at 8 weeks of age and 20 fold normal at 10 months of age. In addition to decreased serum T3 and T4 levels, homozygotes have increased serum thyroid stimulating hormone levels, reduced levels of serum IGFBP-3, IGFBP-4, and IGFBP-2, mild anemia, and hypomyelination restricted to the cerebrum. Tgn^cog is an outwardly recessive mutation but microdissection reveals a heterozygous phenotype as well. Thyrofollicular cells of heterozygotes have swollen protein-containing vesicles similar to but more moderate than those found in homozygotes. Thyroid extracts from homozygotes have an increased percent of protein and heterozygotes have an intermediate percent of protein relative to wild type siblings. (Beamer et al., 1987; Adkison et al., 1990; Sugisaki et al., 1991, 1992, and 1993; Kim et al., 1996 and1998.)

Development
The congenital goiter mutation arose spontaneously in the AKR/J strain (then at generation F131) at The Jackson Laboratory. The mutation was maintained via sibling mating for 9 generations then backcrossed once to AKR/J then sibling mated for 4 generations before being backcrossed to BALB/cBy. A single male at N1F4 was bred to a BALB/cBy female, their heterozygous offspring sibling mated to generate a homozygous N1F1 female that was backcrossed to a BALB/cBy male. Their heterozygous N2 offspring were sibling mated to generate a homozygous male that was backcrossed to a BALB/cBy female. The backcross-intercross breeding scheme was continued from this point on using female BALB/cBy and male homozygotes in the backcross generations until the congenic strain reached N10. This strain was then maintained via sibling mating and in 1995 homozygous males at generation N10F21 were bred with C57BL/6J females to generate embryos for cryopreservation.

Related Strains

Strains carrying   Tg^cog allele

000936

CBy.AK-Tgcog/J

View Strains carrying   Tg^cog     (1 strain)

Phenotype

Phenotype Information

View Research Applications

Research Applications

This mouse can be used to support research in many areas including:

Tg^cog related

Cell Biology Research
Post-translational Processing
Protein Processing

Endocrine Deficiency Research
Thyroid Defects

Internal/Organ Research
Thyroid Defects

Mouse/Human Gene Homologs
congenital goiter with hypothyroidism

Currently there is no phenotype information for this strain.

Genes & Alleles

Gene & Allele Information

Allele Symbol		Tgcog
Allele Name		congenital goiter
Allele Type		Spontaneous
Common Name(s)		Tgncog; cog;
Strain of Origin		AKR/J
Gene Symbol and Name		Tg, thyroglobulin
Chromosome		15
Gene Common Name(s)		AITD3; TGN; Tgn; cog; congenital goiter;
Molecular Note		The mutation is a C to T transition yielding a leucine to proline change at residue 2263. This falls within the acetylcholinesterase domain and impacts protein conformation. This conformational mutation is temperature sensitive; there is an increase in the level of TGN secreted from mutant thyrocytes at 31 degrees relative to the level secreted at 37 degrees, which is below the threshold of detection by PAGE. A small amount of functional TGN is processed in homozygous mice and serum triiodothyroinine and tetraiodothyroinine are found, albeit at vastly reduced levels. [MGI Ref ID J:49474]

Genotyping

Genotyping Information

This strain will not have a genotyping protocol or one is not currently available.

Helpful Links

Optimizing PCR Protocols

References

References

Selected Reference(s)

Beamer WG; Maltais LJ; DeBaets MH; Eicher EM. 1987. Inherited congenital goiter in mice. Endocrinology 120(2):838-40. [PubMed: 3803305]  [MGI Ref ID J:8567]

Additional References

Adkison LR; Taylor S; Beamer WG. 1990. Mutant gene-induced disorders of structure, function and thyroglobulin synthesis in congenital goitre (cog/cog) in mice. J Endocrinol 126(1):51-8. [PubMed: 1696305]  [MGI Ref ID J:46324]

Mayerhofer A; Amador AG; Beamer WG; Bartke A. 1988. Ultrastructural aspects of the goiter in cog/cog mice. J Hered 79(3):200-3. [PubMed: 3392390]  [MGI Ref ID J:24038]

Sugisaki T; Beamer WG; Noguchi T. 1992. Microcephalic cerebrum with hypomyelination in the congenital goiter mouse (cog). Neurochem Res 17(10):1037-40. [PubMed: 1508304]  [MGI Ref ID J:3079]

Tg^cog related

Beamer WG; Coleman DL. 1982. [Adipose storage deficiency (asd)]. Mouse News Lett 67:21.  [MGI Ref ID J:13923]

Kim PS; Hossain SA; Park YN; Lee I; Yoo SE; Arvan P. 1998. A single amino acid change in the acetylcholinesterase-like domain of thyroglobulin causes congenital goiter with hypothyroidism in the cog/cog mouse: a model of human endoplasmic reticulum storage diseases. Proc Natl Acad Sci U S A 95(17):9909-13. [PubMed: 9707574]  [MGI Ref ID J:49474]

Sugisaki T; Beamer WG; Noguchi T. 1992. Microcephalic cerebrum with hypomyelination in the congenital goiter mouse (cog). Neurochem Res 17(10):1037-40. [PubMed: 1508304]  [MGI Ref ID J:3079]

Health & husbandry

Health & Colony Maintenance Information

Currently there no information available for this strain. This may be due to the supply level of this strain.

Purchasing information

Pricing, Supply Level & Notes, Controls, General Terms & Conditions

Pricing

Supply Details

Standard Supply

Cryopreserved. Ready for recovery. Please refer to pricing and supply notes for further information.

Supply Notes

Cryorecovery - Standard. At least two mice that carry the mutation (if it is a mutant strain) will be provided. The total number of animals provided, their gender and genotype will vary. Please inquire if larger numbers of animals with specific genotypes and genders are needed. IMPORTANT NOTE: The genotypes of the animals provided may not reflect the mating scheme utilized by The Jackson Laboratory prior to cryopreservation, or that discussed in the strain description. Please inquire for possible genotypes for this specific strain. Animals typically ship within 13 to 16 weeks from your order. If a second cryorecovery is needed in order to provide the minimum number of animals, animals will typically ship within 25 weeks. Cryorecovery to establish a Dedicated Supply for greater quantities of mice. One to two pairs will be recovered to establish a Dedicated Supply of mice. Price by quotation. For more information on Dedicated Supply, please contact JAX® Services, Tel: 1-800-422-6423 or 1-207-288-5845.

General Terms and Conditions

See Terms of Use

The Jackson Laboratory's Genotype Promise

The Jackson Laboratory has rigorous genetic quality control and mutant gene genotyping programs to ensure the genetic background of JAX^® Mice strains as well as the genotypes of strains with identified molecular mutations. JAX^® Mice strains are only made available to researchers after meeting our standards. However, the phenotype of each strain may not be fully characterized and/or captured in the strain data sheets. Therefore, we cannot guarantee a strain's phenotype will meet all expectations. To ensure that JAX^® Mice will meet the needs of individual research projects or when requesting a strain that is new to your research, we suggest ordering and performing tests on a small number of mice to determine suitability for your particular project.

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