Strain Name: |
WLHR/LeJ |
|---|---|
Stock Number: |
000147 |
Availability: | Repository-Cryopreserved |
General Terms and Conditions |
| Genes & Alleles | Esd; Hr; Hrhr; wl; ; |
Product Information
Strain Details
Type Inbred Strain Additional information on Inbred Strains. Type JAX® GEMM® Strain - Mutant Strain Additional information on JAX® GEMM® Strains. Type Segregating Inbred Species laboratory mouse Appearance
brown, haired, normal gait
Related Genotype: a/a Tyrp1b/Tyrp1b wl + Esdc/+ Hrhr Esdc or a/a Tyrp1b/Tyrp1b ? + Esdc/+ ? Esdc
brown, haired, wobbly gait
Related Genotype: a/a Tyrp1b/Tyrp1b + wl Esdc/? wl Esdc
pigmented, hairless, normal gait
Related Genotype: a/a Tyrp1b/Tyrp1b Hrhr + Esdc/Hrhr ? EsdcImportant Note
This strain is homozygous for Esdc and segregating for Hrhr and wl, which are held in repulsion.Strain Description
WLHR/Le is a balanced stock with wabbler-lethal (wl) and hairless (hr) spontaneous mutations maintained in repulsion on chromosome 14. Homozygous wabbler-lethal mutant mice are first recognizable at 12 days of age and usually die at about 4 weeks. They have an abnormal wobbly gait and a pronounced tremor when walking. In an extensive study of behavioral development of this mutant, homozygous wabbler-lethal mice were shown to be deficient in nearly all behaviors tested. Histological examination showed myelin degeneration widely distributed throughout the CNS, particularly in the vestibulocerebellar and spinocerebellar systems. Electron microscopy showed widespread axonal (Wallerian) degeneration in the medulla with secondary myelin dissolution. Similar abnormalities were present to a lesser extent in the basal ganglia, spinal cord, and cerebellum and in the optic nerve. Homozygous hairless mutant mice have a higher incidence and earlier onset of leukemia, reducible by virus-specific antibody. A deficiency of splenic T helper cells (Ly1+) may account for low cellular immune response of homozygous mutant mice. The coat is normal on hairless mice up to 10 days but then hair is lost from the follicle. Waves of hair growth with few thin fuzzy hairs ocur at monthly intervals for some time but homozygotes eventually become continuously hairless. Vibrissae are repeatedly regrown and shed, becoming more abnormal with age. Toenails are long and curved. There is hyperkeratosis of statified epithelium and the upper part of hair canals beginning at 14 days. Hair club formation is abnormal. Cysts form from the hyperkeratotic upper part of hair canals and sheaths of abnormal follicles stranded in dermis. Some cysts also form from sebaceous glands. All cysts undergo sebaceous transformation and later keratinization.Strain Development
The first wabbler lethal (wl) mouse appeared in descendents of a non-inbred pirouette (pi/pi) mouse carrying brown (Typr1b) and nonagouti (a) that was returned to the Jackson Laboratory from Dr. R. A. Fisher in 1948 after the fire. The pirouette male was mated to a C57BL/6J female and several lines were made. An outcross was also made to strain HD/Hu of Dr. Katrina Hummel. Strain HD/Hu was homozygous for hairless (Hrhr) and dilute (Myo5ad). The stock was bred using tested but non-sibling matings until 1958 when inbreeding was started as a balanced stock with wl and hr in repulsion phase because they were closely linked on chromosome 14. The stock was also selected to be homozygous brown and nonagouti. It was cryopreserved in 1989 by mating known doubly heterozygous males to either known heterozygous or untested females at generations F115-120.
Related Disease (OMIM) Terms |
Mammalian Phenotype Terms assigned by genotype |
Gene & Allele Details
| Allele Symbol | Hrhr | ||
|---|---|---|---|
| Allele Name | hairless | ||
| Common Name(s) | hr; | ||
| Gene Symbol and Name | Hr, hairless | ||
| Chromosome | 14 | ||
| Gene Common Name(s) | ALUNC; AU; HSA277165; N; ba; baldy; bldy; rh; rh-bmh; rhino-bald Mill Hill; | ||
| General Note | Homozygous Hrhr/Hrhr mice develop a normal coat up to the age of about 10 days, but then lose all hair. The complete hair is lost from the follicle, not merely broken off. Waves of hair growth producing a few thin fuzzy hairs occur at intervals of about a month for some time thereafter, but the animals eventually appear continuously hairless (J:2409).The vibrissae are repeatedly regrown and shed, and become more abnormal with age. Toenails are excessively long and curved. There ishyperkeratosis of the stratified epithelium and upper part of the hair canals beginning at about 14 days. Hair club formation is abnormal, with the internal root sheath coalescing around the terminal part of the hair shaft so that the lower part of the external root sheath fails to follow the ascending hair club and becomes stranded in the dermis. Cysts develop from two sources, the hyperkeratotic upper part of the hair canals, and the sheaths of the abnormal follicles stranded in the dermis (J:14940, J:5239). Some cysts arise from isolated sebaceous glands. Regardless of their origin, all cysts undergo a sebaceous transformation and later a keratinization (J:14889).Hairless mice are generally fertile, but most females do not nurse their young well (J:2409).Homozygous hairless mice of the inbred HRS/J strain have a higher incidence of leukemia with earlier onset than their normal sibs, a 13-fold higher titer of ecotropic virus in tail extracts at 6 months of age (J:5726), a 100-fold higher titer of xenotropic virus in the thymus at 8 months of age (J:5908), and a lower cellular immune response to tumor viruses that may be responsible for the increased tumor susceptibility of the mice (J:6535). The incidence of leukemia in Hr homozygotes can be significantly reduced by passive immunization with virus-specific antiserum (J:6059). The lower cellular immune response of these mice is characteristic of spleen cells but not of lymph node cells and is due to a deficiency of T helper (Ly-1+) cells (J:6375, J:6087).The Hrhr mutation was caused by a retroviral integration, as shown by the complete concordance of the mutation and the provirus in several strains carrying Hr and the excision of the provirus in a haired revertant (J:9252). The gene mutated in hr has been identified and cloned. Its predicted protein product has 1182 amino acids, and includes a zinc finger domain. Expression sites are consonant with the sites of abnormalities in hairless mutants (J:19624). The Hrhr mutation disrupts the integrity of tissues in the hair follicle (J:47743). | ||
| Molecular Note | Mice homozygous for hr contain one or more MLV proviruses closely linked to this allele. Molecular analysis of an hr (haired) revertant showed the presence of only a single proviral, MX40, LTR establishing a causal relationship between the viral integration and the hr mutant allele. [J:92053] [J:9252] | ||
| Allele Symbol | wl | ||
| Allele Name | wabbler lethal | ||
| Strain of Origin | STOCK pi | ||
| General Note | Homozygotes are first recognizable at 12 days of age and usually die at about 4 weeks. They have an abnormal wobbly gait and a pronounced tremor when walking. Difficulty in walking increases in severity until the animal dies (J:13068). In an extensive study of behavioral development of this mutant, homozygotes were shown to be deficient in nearly all behaviors tested. From 20 days until death they were hypoglycemic (J:14851). Histological examination showed myelin degeneration widely distributed throughout the central nervous system, particularly in the vestibulocerebellar and spinocerebellar systems (J:15162). Electron microscopy showed widespread axonal (Wallerian) degeneration in the medulla with secondary myelin dissolution. Similar abnormalities were present to a lesser extent in the basal ganglia, spinal cord, and cerebellum (J:5028) and in the optic nerve (J:2856). Primary demyelination was found in the sciatic nerve (J:5028). Aromatic amino acid metabolism is abnormal in affected mice with depressed activity of phenyalanine hydroxylase and elevated activity of several other enzymes. This condition characterizes several other neurological mutants and is probably secondary to their neurological deficit (J:5108). | ||
| Gene Symbol and Name | Esd, esterase D/formylglutathione hydrolase | ||
| Chromosome | 14 | ||
| Gene Common Name(s) | Es-10; Es10; esterase 10; esterase D; | ||
Related Strains
Strains carrying Hrhr allele
001737 B6.A-H2-T18a.HRS-Hrhr/J 002922 D2.HRS-Hrhr/J 000673 HRS/J 001103 HRS/J-Hrhr Esdb/+ Esdb/J 002335 SKH2/J View Strains carrying Hrhr (5 strains)
004835 B6 x B6JCu.Cg-wl/J
Additional Web Information
JAX Notes, January 1989; 436. Phenotypic abnormalities in hr-locus mutants.
Research Applications
This mouse can be used to support research in many areas including:
Hrhr relatedMetabolism Research
wl relatedCancer Research
Increased Tumor Incidence (Leukemia: lymphocytic)
Increased Tumor Incidence (Lymphomas: thymic)
Increased Tumor Incidence (Skin Cancers: Induced)
Toxicology
Cardiovascular Research
Diet-Induced Atherosclerosis (Relatively Resistant)
Dermatology Research
Skin and Hair Texture Defects
Immunology and Inflammation Research
Immunodeficiency Associated with Other Defects
Research Tools
Toxicology Research (drug/compound testing)
Neurobiology Research
Ataxia (Movement) Defects
Myelination Defects
Tremor Defects
Vestibular and Hearing Defects
Sensorineural Research
Vestibular and Hearing Defects
References
Additional ReferencesPrice and Supply Information
| Strain Name: | WLHR/LeJ |
| Stock Number: | 000147 |
Price Details
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Supply Details
| Standard Supply | Repository-Cryopreserved. Must Be Recovered. Please refer to the Supply Notes for further information. |
|---|---|
| Supply Notes |
Cryorecovery of Strains Needing Progeny Testing. The recovery process begins when a signed agreement form is returned to the Customer Service Department after order placement. Although results vary by strain, at least two untested males and two untested females (two pairs) will be recovered, typically within 15 weeks of our receipt of the signed agreement form. If the first recovery attempt is unsuccessful or only one pair is recovered, a second recovery will be done, extending the overall recovery time to approximately 25 weeks. However, all pups recovered will be sent. Progeny testing is required to identify the genotype of mice of this strain, as a genotyping assay is not available. This type of testing involves breeding the recovered animals and assessing the phenotype of the offspring in order to identify animals carrying the mutation of interest. We can perform the progeny testing for you as a service or we can ship all recovered animals (at least two untested pairs) to you for progeny testing at your facility. If you perform the progeny testing, there is NO guarantee that a carrier will be identified. If we perform progeny testing as a service, additional breeding time will be required. In this case, when a male and female (one pair) are identified that carry the mutation, they and their offspring will be shipped. Delivery time for strains requiring progeny testing often exceeds 25 weeks and may take 12 months or more due to the difficulties in breeding some strains. The progeny testing cost is in addition to the recovery cost and is based on the number of boxes used and the time taken to produce the mice identified as carrying the mutation. Please note that identified pairs may not reflect the mating scheme utilized by The Jackson Laboratory prior to cryopreservation of the strain. Mating schemes are sometimes modified for successful cryopreservation. Please contact Customer Service for more information on the cost of progeny testing for a strain: Tel: 1-800-422-6423 or 1-207-288-5845. Cryorecovery to establish a Dedicated Supply for greater quantities of mice |
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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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