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- Description
- Phenotype
- Genes & alleles
- Genotyping
- Health & husbandry
- References
- Purchasing information
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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
Type Mutant Stock; Spontaneous Mutation; Additional information on Genetically Engineered and Mutant Mice. Visit our online Nomenclature tutorial. Species laboratory mouse Generation N28 F1 Appearance
black
Related Genotype: a/aDescription
Mice homozygous for the wasted spontaneous mutation (Eef1a2wst) can be recognized at 20 days of age by tremor and uncoordinated body movements. Homozygous mutant mice develop progressive paralysis and do not survive beyond 30 days. They have marked lymphoid hypoplasia in the spleen, thymus, lymph nodes, and peripheral blood. Wasted mice have a severely reduced number of IgA plasma cells in the entire large and small intestines, but the number of such cells in the spleen and the level of serum IgA are normal.Neuronal degeneration occurs in the brain and spinal cord. Spinal cord abnormalities are preceded by gliosis following along a rostrocaudal gradient. Thoracic/abdominal muscles exhibit a progressive denervation of muscle fibers, with progressive asynchronous retraction of motor nerve terminals. (Newbery HJ, et al. 2005)
Development
The wasted mutation arose spontaneously on the HRS/J background in 1972 and was subsequently maintained by breeding hosts of homozygous ovarian transplants to B6C3Fe-a/a F1 then intercrossing the heterozygous offspring. In 1987 heterozygotes at generation N28F1 were intercrossed to generate embryos for cryopreservation.
Control Information
| Control | ||
|---|---|---|
| Untyped from the colony | ||
| Considerations for Choosing Controls | ||
Related Strains
Strains carrying a allele
View Strains carrying a (104 strains)
Phenotype
Phenotype Information
assigned by genotype
Eef1a2wst/Eef1a2wst
B6C3Fe a/a-Eef1a2wst/J
- behavior/neurological phenotype
- tremors (MGI Ref ID J:24753)
- fine intention tremor over the entire body is visible by approximately 20 days of age
- weakness (MGI Ref ID J:24753)
- increasing flacidity of the hind legs and an overall debilitated appearance follow the onset of tremor at 20 days of age
- nervous system phenotype
- Purkinje cell degeneration (MGI Ref ID J:6766)
- evident by 22 to 29 days of age
- demyelination (MGI Ref ID J:6766)
- at 22 to 29 days of age focal demyelination is found in the ventral columns of the spinal cord and in the cerebellar cortex
- immune system phenotype
- decreased plasma cell number (MGI Ref ID J:7903)
- although the intestinal villi are normal in size, there is a progressive decline in gut IgA plasma cells and no detectable IgA plasma cells in the gut lamina propria and submucosae by 27 days of age
- decreased spleen weight (MGI Ref ID J:6766)
- consistent with lymphoid hypoplasia the ratio of spleen weight to body weight is less than half that of normal
- decreased susceptibility to type IV hypersensitivity reaction (MGI Ref ID J:6766)
- less footpad inflammation than heterozygous controls in response to injected sheep red blood cells 4 days after priming
- decreased thymus weight (MGI Ref ID J:6766)
- consistent with lymphoid hypoplasia the ratio of thymic weight to body weight is much smaller than normal
- lymphoid hypoplasia (MGI Ref ID J:6766)
- by 28 days of age homozygotes have a threefold diminution in the number of circulating leukocytes, decreased ratios of spleen, thymus, and lymph nodes to body weight, few and small Peyer's patches, reduction in cellularity of the thymic cortex, and poorly developed follicles in the lymph nodes and splee
- the percentages of T and B cells is normal with the lymphoid hypoplasia not being restricted to a lymphocyte subpopulation
- small lymph nodes (MGI Ref ID J:6766)
- consistent with lymphoid hypoplasia the ratio of brachial lymph node weight to body weight is much smaller than normal
- cellular phenotype
- chromosomal instability (MGI Ref ID J:6766)
- bone marrow cells from 23 to 28 day old homozygotes shows fourfold greater incidence of chromosomal damage, such as chromatid breaks, gaps, and fragments, than is found in heterozygous littermate controls
- increased cellular sensitivity to gamma-irradiation (MGI Ref ID J:6766)
- 2 of 2 homozygotes exposed to 220R from a cesium irradiator then treated with colchicine have chromosomal damage in 80% of 30 metaphase cells from bone marrow cell suspensions while controls have only monor damage in 30% of the cells scored
- life span-post-weaning/aging
- premature death (MGI Ref ID J:24753)
- average age of death is 28 days
- growth/size phenotype
- postnatal slow weight gain (MGI Ref ID J:6766)
- no weight increase after 20 days of age
- weight loss (MGI Ref ID J:24753)
- at 24 days of age there is a sudden onset emaciated appearance from the ribs caudally
- hematopoietic system phenotype
- decreased plasma cell number (MGI Ref ID J:7903)
- although the intestinal villi are normal in size, there is a progressive decline in gut IgA plasma cells and no detectable IgA plasma cells in the gut lamina propria and submucosae by 27 days of age
- decreased spleen weight (MGI Ref ID J:6766)
- consistent with lymphoid hypoplasia the ratio of spleen weight to body weight is less than half that of normal
- decreased thymus weight (MGI Ref ID J:6766)
- consistent with lymphoid hypoplasia the ratio of thymic weight to body weight is much smaller than normal
The following phenotype information may relate to a genetic background differing from this JAX® Mice strain.
Eef1a2wst/Eef1a2wst
involves: HRS/J
- muscle phenotype
- decreased muscle weight (MGI Ref ID J:104855)
- by 20 days of age there is a decline in muscle mass and by 27 days of age the ratio of muscle to body weight is 1.49 compared with 4.44 in heterozygotes
- nervous system phenotype
- abnormal endplate potential (MGI Ref ID J:104855)
- at 23 days of age 20% of neuromuscular junctions in transverse abdominus muscles show weak synaptic transmission with random failures
- evoked endplate potentials in flexor digitorum brevis muscles do not show weak synaptic transmission until 25 days of age so there is progressive rostrocaudal deterioration in synaptic transmission
- abnormal motor neuron morphology (MGI Ref ID J:104855)
- in addition to vacuolation, an abnormal accumulation of phosphorylated neurofilaments is found in the motor neurons of the anterior horn of the cervical spinal cord
- motor neuron degeneration (MGI Ref ID J:104855)
- abnormal muscle innervation (MGI Ref ID J:104855)
- there is a rostrocaudal progressive retraction of motor nerve terminals
- abnormal neuromuscular synapse morphology (MGI Ref ID J:104855)
- as early as 17 days of age a small population of motor endplates is found to have no overlying motor nerve terminal, and this progresses such that by 27 days of age approximately 42% of endplates have no overlying motor nerve terminal
- abnormal neurotransmitter uptake (MGI Ref ID J:104855)
- FM1-43 and TRITC alpha bungarotoxin staining shows diminished uptake in a majority of endplates of transversus abdominus muscles from 25 day old homozygotes
- abnormal spinal cord ventral horn morphology (MGI Ref ID J:104855)
- at 28 days of age, but not 24 days of age, vacuolation is found in the anterior horn cells, but not at the lumbar level of the spinal cord
- decreased neurotransmitter release (MGI Ref ID J:104855)
- diminished spontaneous miniature endplate potentials in transversus abdominus muscles at 25 days of age
- gliosis (MGI Ref ID J:104855)
- although there is no evidence even at 29 days of age of microgliosis by CD45 staining, strong foci of GFAP staining in the anterior horn shows cervical, and to a lesser degree thoracic and lumbar, gliosis as early as 19 days of age
- behavior/neurological phenotype
- impaired coordination (MGI Ref ID J:104855)
- from 21 days of age on homozygotes are progressively less able to stay on a rotarod for as long as normal littermate controls
Eef1a2wst/Eef1a2wst
Background Not Specified
- hematopoietic system phenotype
- thymus atrophy (MGI Ref ID J:50410)
- at 27 days of age the thymus is less than half the normal diameter, has a substantial increase in the number of apoptotic cells, but near-normal proportions of thymocyte subpopulations
- immune system phenotype
- thymus atrophy (MGI Ref ID J:50410)
- at 27 days of age the thymus is less than half the normal diameter, has a substantial increase in the number of apoptotic cells, but near-normal proportions of thymocyte subpopulations
This mouse can be used to support research in many areas including:Eef1a2wst related
Immunology and Inflammation Research
Immunodeficiency Associated with Other Defects
Internal/Organ Research
Lymphoid Tissue Defects
Spleen Defects
Neurobiology Research
Neuromuscular Defects
Genes & Alleles
Gene & Allele Information
| Allele Symbol | Eef1a2wst | ||
|---|---|---|---|
| Allele Name | wasted | ||
| Allele Type | Spontaneous | ||
| Common Name(s) | wst; | ||
| Strain of Origin | HRS/J | ||
| Gene Symbol and Name | Eef1a2, eukaryotic translation elongation factor 1 alpha 2 | ||
| Chromosome | 2 | ||
| Gene Common Name(s) | EEF1AL; EF-1-alpha-2; EF1A; Eef1a; FLJ41696; HS1; Ps10; RATPS10; S1; STN; STNL; wasted; wst; | ||
| General Note | The enzymatic specific activity of ADA (which takes into account the reduced tissue weights of wasted mice) is increased in the spleen and cerebellum but decreased in the thymus of these mutants (J:12932). Homozygotes are recognized at about 20 days witha tremor, uncoordinated body movements, and the beginning of body weight loss. They develop progressive paralysis and do not survive beyond 30 days of age (J:6766). Wasted mutant mice have marked lymphoid hypoplasia in the spleen, thymus, lymph nodes,and peripheral blood. Thymocytes from these animals show an increase of in situ apoptosis, which probably accounts for the thymus hypoplasia (J:50410). The relative proportion of T and B cells is not affected. In the central nervous system there is degeneration of Purkinje cells and focal demyelination in the cerebellar cortex and ventral columns of the spinal cord (J:6766). Wasted mice show some similarities to human ataxia telangiectasia (AT; MIM 208900; human gene, ATM; mouse gene, Atm) (J:6766),but there are also numerous differences. Eef1a2ws mice have a marked increase in spontaneous and gamma radiation-induced chromosomal abnormalities in bone marrow, as there is in AT (J:6766, J:8276). But the mutant mice lack the increased sensitivity of spleen cells to killing by ultraviolet radiation (J:20567), the increased sensitivity of cultured fibroblasts to killing by X or gamma radiation (J:8348, J:13960), and the increased post-irradiation inhibition of DNA synthesis (J:7561) characteristic of AT. In addition, the immunological defects of wasted mice differ considerably from those in human AT (J:8288). Wasted mice have a severely reduced number of IgA plasma cells in the entire large and small intestine, but the level of such cellsin spleen and the level of serum IgA are normal (J:7903). The mutants also show an extensive cytokine imbalance of many immunologically relevant gene products (IL5, IL1, IL2, IFNG, and TGFB1) which may trigger the wasted pathogenesis (J:19431). The overlapping similarities between AT and wasted, however, have suggested the possibility that Eef1a2 and Atm may be involved in a common pathway, such as a signal-transduction pathway that regulates protein synthesis (J:48050). In fact Eef1a2ws mice show abnormal expression of the proliferating cellular nuclear antigen (PCNA) in the thymus (J:37955). | ||
| Molecular Note | The mutation is a 15.8kb deletion that removes the promoter region and first noncoding exon of the gene. [MGI Ref ID J:47466] | ||
| Allele Symbol | a | ||
| Allele Name | nonagouti | ||
| Allele Type | Spontaneous | ||
Genotyping
Genotyping Information
Genotyping Protocols
Eef1a2wst, Separated PCR
Helpful Links
Genotyping resources and troubleshooting
References
References
Additional References
Kaiserlian D; Delacroix D; Bach JF. 1985. The wasted mutant mouse. I. An animal model of secretory IgA deficiency with normal serum IgA. J Immunol 135(2):1126-31. [PubMed: 3874230] [MGI Ref ID J:7903]
Pan J; Ruest LB; Xu S; Wang E. 2004. Immuno-characterization of the switch of peptide elongation factors eEF1A-1/EF-1alpha and eEF1A-2/S1 in the central nervous system during mouse development. Brain Res Dev Brain Res 149(1):1-8. [PubMed: 15013623] [MGI Ref ID J:88820]
Shultz LD; Sweet HO; Davisson MT; Coman DR. 1982. 'Wasted', a new mutant of the mouse with abnormalities characteristic to ataxia telangiectasia. Nature 297(5865):402-4. [PubMed: 7078649] [MGI Ref ID J:6766]
Eef1a2wst relatedAbbott CM; Skidmore CJ; Searle AG; Peters J. 1986. Deficiency of adenosine deaminase in the wasted mouse. Proc Natl Acad Sci U S A 83(3):693-5. [PubMed: 3456164] [MGI Ref ID J:8181]
Chambers DM; Peters J; Abbott CM. 1998. The lethal mutation of the mouse wasted (wst) is a deletion that abolishes expression of a tissue-specific isoform of translation elongation factor 1alpha, encoded by the Eef1a2 gene. Proc Natl Acad Sci U S A 95(8):4463-8. [PubMed: 9539760] [MGI Ref ID J:47466]
Geiger JD; Nagy JI. 1986. Lack of adenosine deaminase deficiency in the mutant mouse wasted. FEBS Lett 208(2):431-4. [PubMed: 3780980] [MGI Ref ID J:12932]
Hafezparast M; Fisher E. 1998. Wasted by an elongation factor. Trends Genet 14(6):215-7. [PubMed: 9635401] [MGI Ref ID J:48050]
Inoue T; Aikawa K; Tezuka H; Kada T; Shultz LD. 1986. Effect of DNA-damaging agents on isolated spleen cells and lung fibroblasts from the mouse mutant wasted, a putative animal model for ataxia-telangiectasia. Cancer Res 46(8):3979-82. [PubMed: 2425937] [MGI Ref ID J:8348]
Kaiserlian D; Delacroix D; Bach JF. 1985. The wasted mutant mouse. I. An animal model of secretory IgA deficiency with normal serum IgA. J Immunol 135(2):1126-31. [PubMed: 3874230] [MGI Ref ID J:7903]
Kaiserlian D; Savino W; Uriel J; Hassid J; Dardenne M; Bach JF. 1986. The wasted mutant mouse. II. Immunological abnormalities in a mouse described as a model of ataxia-telangiectasia. Clin Exp Immunol 63(3):562-9. [PubMed: 2423278] [MGI Ref ID J:8288]
Khalyfa A; Bourbeau D; Chen E; Petroulakis E; Pan J; Xu S; Wang E. 2001. Characterization of elongation factor-1A (eEF1A-1) and eEF1A-2/S1 protein expression in normal and wasted mice. J Biol Chem 276(25):22915-22. [PubMed: 11294870] [MGI Ref ID J:70131]
Lee S; Ann DK; Wang E. 1994. Cloning of human and mouse brain cDNAs coding for S1, the second member of the mammalian elongation factor-1 alpha gene family: analysis of a possible evolutionary pathway. Biochem Biophys Res Commun 203(3):1371-7. [PubMed: 7945283] [MGI Ref ID J:20567]
Libertin CR; Ling-Indeck L; Padilla M; Woloschak GE. 1994. Cytokine and T-cell subset abnormalities in immunodeficient wasted mice. Mol Immunol 31(10):753-9. [PubMed: 8035837] [MGI Ref ID J:19431]
Luo C; Copeland NG; Jenkins NA; Edelhoff S; Disteche C; Hogan PG; Rao A. 1996. Normal function of the transcription factor NFAT1 in wasted mice. Chromosome localization of NFAT1 gene. Gene 180(1-2):29-36. [PubMed: 8973343] [MGI Ref ID J:37134]
Newbery HJ; Gillingwater TH; Dharmasaroja P; Peters J; Wharton SB; Thomson D; Ribchester RR; Abbott CM. 2005. Progressive loss of motor neuron function in wasted mice: effects of a spontaneous null mutation in the gene for the eEF1 A2 translation factor. J Neuropathol Exp Neurol 64(4):295-303. [PubMed: 15835265] [MGI Ref ID J:104855]
Newbery HJ; Loh DH; O'Donoghue JE; Tomlinson VA; Chau YY; Boyd JA; Bergmann JH; Brownstein D; Abbott CM. 2007. Translation elongation factor eEF1A2 is essential for post-weaning survival in mice. J Biol Chem 282(39):28951-9. [PubMed: 17640869] [MGI Ref ID J:125352]
Nordeen SK; Schaefer VG; Edgell MH; Hutchison CA 3d; Shultz LD; Swift M. 1984. Evaluations of wasted mouse fibroblasts and SV-40 transformed human fibroblasts as models of ataxia telangiectasia in vitro. Mutat Res 140(4):219-22. [PubMed: 6206393] [MGI Ref ID J:7561]
Potter M; Bernstein A; Lee JM. 1998. The wst gene regulates multiple forms of thymocyte apoptosis. Cell Immunol 188(2):111-7. [PubMed: 9756641] [MGI Ref ID J:50410]
Shultz LD; Sweet HO; Davisson MT; Coman DR. 1982. 'Wasted', a new mutant of the mouse with abnormalities characteristic to ataxia telangiectasia. Nature 297(5865):402-4. [PubMed: 7078649] [MGI Ref ID J:6766]
Sweet HO. 1981. Wasted (wst) Mouse News Lett 65:27. [MGI Ref ID J:24753]
Tezuka H; Inoue T; Noguti T; Kada T; Shultz LD. 1986. Evaluation of the mouse mutant wasted as an animal model for ataxia telangiectasia. I. Age-dependent and tissue-specific effects. Mutat Res 161(1):83-90. [PubMed: 3702898] [MGI Ref ID J:8276]
Thacker J; Masson W. 1984. Radiation sensitivity of cells cultured from 'wasted` mice Mouse News Lett 70:80. [MGI Ref ID J:13960]
Woloschak GE; Paunesku T; Libertin CR; Chang-Liu CM; Churchill M; Panozzo J; Grdina D; Gemmell MA; Giometti C. 1996. Regulation of thymus PCNA expression is altered in radiation-sensitive wasted mice. Carcinogenesis 17(11):2357-65. [PubMed: 8968049] [MGI Ref ID J:37955]
van Buul PP; Tuinenburg-Bol Raap A; Goudzwaard HJ; Seelen CM; Beechey CV; Natarajan AT; Searle AG. 1991. Cytogenetic characterization of radiosensitive mouse mutants. Mutat Res 251(2):171-9. [PubMed: 1720867] [MGI Ref ID J:4646]
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
| Pricing for USA, Canada and Mexico shipping destinations |
|
Animals Provided
Price (US dollars $) Cryorecovery Fee $1900.00 At least two mice that carry the mutation (if it is a mutant strain) will be provided. Their genotypes may not reflect those discussed in the strain description. Please inquire for possible genotypes and see additional details below.
Additional Supply Details
| Pricing for International shipping destinations |
|
Animals Provided
Price (US dollars $) Cryorecovery Fee $2470.00 At least two mice that carry the mutation (if it is a mutant strain) will be provided. Their genotypes may not reflect those discussed in the strain description. Please inquire for possible genotypes and see additional details below.
Additional Supply Details
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Supply Details
| Standard Supply | Cryopreserved. Ready for recovery. Please refer to pricing and supply notes for further information. |
|---|---|
| Supply Notes |
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Control Information
| Control | ||
|---|---|---|
| Untyped from the colony | ||
| Considerations for Choosing Controls | ||
| USA, Canada and Mexico - Control Pricing Information for Genetically Engineered Mutant Strains. | ||
| International - Control Pricing Information for Genetically Engineered Mutant Strains. | ||
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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.Ordering and Purchasing Information
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