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| These BChE (butyrylcholinesterase) mutant mice are a model for human butyrylcholinesterase deficiency and may be useful for studying metabolic effects of organophosphorus toxicants or nerve agents, neurotransmitter function, and anti-Alzheimer's drug therapies. | ||||||||||||||
Type Coisogenic; Mutant Strain; Targeted Mutation; Additional information on Genetically Engineered and Mutant Mice. Visit our online Nomenclature tutorial. Species laboratory mouse Donating Investigator Oksana Lockridge, Eppley Inst. (Univ of Nebraska Med Cntr) Description
Mice homozygous for this BChE mutant allele are viable and fertile with no reported spontaneous abnormalities. All tissues and plasma from homozygous mice are devoid of BChE activity. As BChE (butyrylcholinesterase) is a bioscavenger molecule protecting acetylcholinesterase (AChE) activity against nerve agents and organophosphates, homozygous BChE-deficiency leads to impaired protection from toxic compounds. These BChE mutant mice are a model for human butyrylcholinesterase deficiency and may be useful for studying metabolic effects of organophosphorus toxicants or nerve agents, neurotransmitter function, and anti-Alzheimer's drug therapies.Of note, the donating investigator has multiple strains available that may be useful for testing toxic compounds, including the AChE-deficient (Stock No. 005987), G117H BChE transgenic (Stock No. 007577), and BChE-deficient (see Stock No. 008077 and Stock No. 008087) mice.
Development
The targeting vector was designed to replace the sequence containing the splice junction between intron 1 and exon 2 through the entire signal peptide (including the translation start site and the first 102 amino acids of the encoded BChE protein) with a neomycin cassette. The construct was electroporated into 129S1/Sv-derived CJ7 embryonic stem (ES) cells. Correctly targeted ES cells were injected into recipient blastocysts. The resulting chimeric animals were crossed to 129S1/SvImJ inbred mice. Mutant mice were backcrossed to 129S1/SvImJ for more than 10 generations prior to arrival at The Jackson Laboratory.
| Control | ||
|---|---|---|
| 002448 129S1/SvImJ | ||
| Considerations for Choosing Controls | ||
Strains carrying Bchetm1Loc allele
008087 B6.129S1-Bchetm1Loc/J View Strains carrying Bchetm1Loc (1 strain)
View Related Disease (OMIM) Terms
Related Disease (OMIM) Terms
Butyrylcholinesterase; BCHE - Models with phenotypic similarity to human disease where etiologies involve orthologs.1
1 Human genes are associated with this disease. Orthologs of those genes appear in the mouse genotype(s).
View Mammalian Phenotype Terms
Mammalian Phenotype Terms
assigned by genotype
Bchetm1Loc/Bche+
involves: 129S1/Sv
- life span-post-weaning/aging
- increased sensitivity to xenobiotic induced morbidity/mortality (MGI Ref ID J:130048)
- donepezil-treated mice die within 24 hours unlike similarly treated controls
- (-)-huperzin A-treated mice die within 24 hours unlike similarly treated controls
- homeostasis/metabolism phenotype
- increased physiological sensitivity to xenobiotics (MGI Ref ID J:130048)
- treatment with donepezil induces intermediate symptoms with all mice dying or moribund within 24 hours unlike similarly treated wild-type mice that recover within 24 hours
- donepezil-treated mice fail to recover normal body temperature unlike similarly treated wild-type mice
- (-)-huperzin A-treated mice exhibit protosis, salivation, and lacrimation with death occurring within 24 hours of treatment unlike similarly treated mice
- increased sensitivity to xenobiotic induced morbidity/mortality (MGI Ref ID J:130048)
- donepezil-treated mice die within 24 hours unlike similarly treated controls
- (-)-huperzin A-treated mice die within 24 hours unlike similarly treated controls
Bchetm1Loc/Bchetm1Loc
involves: 129S1/Sv
- life span-post-weaning/aging
- increased sensitivity to xenobiotic induced morbidity/mortality (MGI Ref ID J:130048)
- donepezil-treated mice die within 3 hours unlike similarly treated controls
- (-)-huperzin A-treated mice die within 10 minutes unlike similarly treated controls
- reproductive system phenotype
- *normal* reproductive system phenotype (MGI Ref ID J:124975)
- mice are fertile
- homeostasis/metabolism phenotype
- increased physiological sensitivity to xenobiotics (MGI Ref ID J:130048)
- treatment with donepezil induces clonic, jerking convulsions and death within 3 hours unlike similarly treated wild-type mice that recover within 24 hours
- (-)-huperzin A-treated mice exhibit protosis, salivation, and lacrimation with death occurring within 10 minutes of treatment unlike similarly treated mice
- however, mice exhibit a normal reaction to CPO and echothiophate
- increased sensitivity to xenobiotic induced morbidity/mortality (MGI Ref ID J:130048)
- donepezil-treated mice die within 3 hours unlike similarly treated controls
- (-)-huperzin A-treated mice die within 10 minutes unlike similarly treated controls
View Research Applications
Research Applications
This mouse can be used to support research in many areas including:
Bchetm1Loc relatedCancer Research
Toxicology
Metabolism Research
Neurobiology Research
Neuromuscular Defects
Neurotransmitter Receptor and Synaptic Vesicle Defects
Receptor Defects
cholinergic receptor
Tremor Defects
Research Tools
Cancer Research
Metabolism Research
Neurobiology Research
Sensorineural Research
Toxicology Research
drug metabolism
drug/compound testing
Neurobiology Research
Alzheimer's Disease
| Allele Symbol | Bchetm1Loc | ||
|---|---|---|---|
| Allele Name | targeted mutation 1, Oksana Lockridge | ||
| Allele Type | Targeted (knock-out) | ||
| Common Name(s) | BChE KO; BChE-; | ||
| Mutation Made By | Oksana Lockridge, Eppley Inst. (Univ of Nebraska Med Cntr) | ||
| Strain of Origin | 129S1/Sv-Oca2<+> Tyr<+> Kitl<+> | ||
| ES Cell Line Name | CJ7 | ||
| ES Cell Line Strain | 129S1/Sv-Oca2<+> Tyr<+> Kitl<+> | ||
| Gene Symbol and Name | Bche, butyrylcholinesterase | ||
| Chromosome | 3 | ||
| Gene Common Name(s) | C730038G20Rik; CHE1; E1; RIKEN cDNA C730038G20 gene; | ||
| Molecular Note | 891bp of the gene is replaced with a neomycin selection cassette, including 485bp from the 3' end of intron 1 and 406bp from the 5' end of exon 2. The region encoding the entire signal peptide, the translation start site, and the first 102 amino acids ofthe mature protein is deleted. [MGI Ref ID J:124975] | ||
Genotyping Protocols
Bchetm1Loc, Standard PCR
Helpful Links
Genotyping resources and troubleshooting
Duysen EG; Li B; Darvesh S; Lockridge O. 2007. Sensitivity of butyrylcholinesterase knockout mice to (--)-huperzine A and donepezil suggests humans with butyrylcholinesterase deficiency may not tolerate these Alzheimer's disease drugs and indicates butyrylcholinesterase function in neurotransmission. Toxicology 233(1-3):60-9. [PubMed: 17194517] [MGI Ref ID J:130048]
Li B; Duysen EG; Saunders TL; Lockridge O. 2006. Production of the butyrylcholinesterase knockout mouse. J Mol Neurosci 30(1-2):193-5. [PubMed: 17192674] [MGI Ref ID J:124975]
Bchetm1Loc relatedDuysen EG; Li B; Lockridge O. 2009. The butyrylcholinesterase knockout mouse a research tool in the study of drug sensitivity, bio-distribution, obesity and Alzheimer's disease. Expert Opin Drug Metab Toxicol 5(5):523-8. [PubMed: 19416087] [MGI Ref ID J:148458]
Colony Maintenance
Breeding & Husbandry When maintaining a live colony, these mice may be bred as heterozygotes or homozygotes.
| Pricing for USA, Canada and Mexico shipping destinations |
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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.
| 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.
| Standard Supply | Cryopreserved. Ready for recovery. Please refer to pricing and supply notes for further information. |
|---|---|
| Supply Notes |
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| Control | ||
|---|---|---|
| 002448 129S1/SvImJ | ||
| 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. | ||
Purchasing Information
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Contact Information
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Technical Support Email Form
| phone: | 207-288-6470 |
| fax: | 207-288-6655 |
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