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Type Congenic; Mutant Strain; Targeted Mutation; Additional information on Genetically Engineered Mutant Mice. Species laboratory mouse Background Strain C57BL/6 Donor Strain 129X1 x 129S1 via R1 (+Kitl-SlJ) ES cell line Generation N9 Donating Investigator John Lazo, University of Pittsburgh Description
Bleomycin (BLM) is a clinically used glycopeptide anticancer agent. It is deaminated in vitro by BLMH. Blmh null mice have decreased viability and fertitility. Only about 65% of the expected number survive the neonatal period. Mice lacking Blmh exhibit variably penetrant tail dermatitis that resembles rodent ringtail. This resembles skin lesions in humans with pellagra, necrolytic migratory erythema, and acrodermatitis enteropathica. Null mice also are more sensitive to acute BLM lethality and develop pulmonary fibrosis following BLM treatment.
| Control | ||
|---|---|---|
| Wild-type from the colony | ||
| 000664 C57BL/6J | ||
| Considerations for Choosing Controls | ||
Congenic Nomenclature
View Mammalian Phenotype Terms
Mammalian Phenotype Terms
assigned by genotype
The following phenotype information may relate to a genetic background differing from this JAX® Mice strain.
Blmhtm1Geh/Blmhtm1Geh
either: (involves: 129S1/Sv * 129S6/SvEvTac * 129X1/SvJ) or (involves: 129S1/Sv * 129X1/SvJ * C57BL/6J)
- lethality-prenatal/perinatal
- neonatal lethality (MGI Ref ID J:54585)
- approximately 36% of homozygotes died as neonates; those surviving the neonatal period were fertile and appeared healthy
- behavior/neurological phenotype
- *normal* behavior/neurological phenotype (MGI Ref ID J:54585)
- the skin lesions were not caused by aggressive behavior: both male and female mutants were housed individually without resolution of the tail dermatitis
- cardiovascular system phenotype
- *normal* cardiovascular system phenotype (MGI Ref ID J:54585)
- homozygotes did not display angitis or vascular thrombosis
- growth/size phenotype
- decreased body size (MGI Ref ID J:54585)
- homozygotes frequently appeared smaller than wild-type or heterozygous littermates
- homeostasis/metabolism phenotype
- increased sensitivity to xenobiotics (MGI Ref ID J:54585)
- homozygotes were hypersensitive to the deleterious effects of bleomycin
- bleomycin doses that were without effect in wild-type mice elicited either pulmonary fibrosis (25 mg/kg) or lethality (50 or 100 mg/kg) in homozygous null mice
- limbs/digits/tail phenotype
- abnormal tail morphology (MGI Ref ID J:54585)
- the gross tail lesions resembled rodent ringtail, and were frequently resolved by weaning
- skin/coat/nails phenotype
- dermatitis (MGI Ref ID J:54585)
- dermatitis on the tail only
- mutants without dermatitis on the tail develop tail dermatitis when kept in a low-humidity atmosphere
- scaly skin (MGI Ref ID J:54585)
- most newborns appeared normal except for a dermatopathy presenting initially as a mild ichthyosis with generalized scaling and sloughing of the skin over the entire body
- at ~7-10 days, the scaling became resolved except on the tail
- no abnormalities were observed in other tissues
- thickened epidermis (MGI Ref ID J:54585)
- similar to ringtail, the tail dermatitis included thickening of the epidermis (parakeratotic hyperkeratosis and acanthosis), areas of necrosis, and neutrophil infiltrate
- unlike ringtail, ballooning degeneration of the upper stratum spinosum and palor were observed in some mutant tails
- immune system phenotype
- dermatitis (MGI Ref ID J:54585)
- dermatitis on the tail only
- mutants without dermatitis on the tail develop tail dermatitis when kept in a low-humidity atmosphere
View Research Applications
Research Applications
This mouse can be used to support research in many areas including:Blmhtm1Geh related
Cancer Research
Growth Factors/Receptors/Cytokines
Increased Tumor Incidence (Lymphomas)
Increased Tumor Incidence (Other Tissues/Organs)
Toxicology
Dermatology Research
Skin and Hair Texture Defects
Developmental Biology Research
Skin and Hair Texture Defects
Endocrine Deficiency Research
Skin Defects
Immunology and Inflammation Research
Inflammation
Internal/Organ Research
Lung Defects
Mouse/Human Gene Homologs
Alzheimer's
Neurobiology Research
Neurodegeneration
Research Tools
Cancer Research
Dermatology Research
Metabolism Research
Toxicology Research (drug metabolism)
| Allele Symbol | Blmhtm1Geh | ||
|---|---|---|---|
| Allele Name | targeted mutation 1, Gregg E Homanics | ||
| Allele Type | Targeted (knock-out) | ||
| Common Name(s) | Bh-; Blmhtm2Geh; | ||
| Mutation Made By | Donald Schwartz, Wayne State Univ School of Medicine | ||
| Strain of Origin | (129X1/SvJ x 129S1/Sv)F1-Kitl+ | ||
| ES Cell Line Name | R1 | ||
| ES Cell Line Strain | (129X1/SvJ x 129S1/Sv)F1-Kitl<+> | ||
| Gene Symbol and Name | Blmh, bleomycin hydrolase | ||
| Chromosome | 11 | ||
| Gene Common Name(s) | AI035728; BH; BMH; MGC:36899; MGC:37104; MGC:39026; expressed sequence AI035728; | ||
| Molecular Note | A genomic fragment containing exon 3 and flanking DNA was replaced with a neomycin selection cassette. The deleted sequences include the region encoding the catalytic cysteine of the protein. Northern blot analysis on total embryonic RNA derived from homozygous mice demonstrated that no transcript was produced from this allele. Western blot and enzymatic activity assays confirmed that no functional protein was made in homozygous mice. [MGI Ref ID J:54585] | ||
Genotyping Protocols
Blmhtm1Geh, STD PCR, vers. 2
Helpful Links
Optimizing PCR Protocols
Blmhtm1Geh relatedMontoya SE; Thiels E; Card JP; Lazo JS. 2007. Astrogliosis and behavioral changes in mice lacking the neutral cysteine protease bleomycin hydrolase. Neuroscience 146(3):890-900. [PubMed: 17391860] [MGI Ref ID J:122060]
Schwartz DR; Homanics GE; Hoyt DG; Klein E; Abernethy J; Lazo JS. 1999. The neutral cysteine protease bleomycin hydrolase is essential for epidermal integrity and bleomycin resistance. Proc Natl Acad Sci U S A 96(8):4680-5. [PubMed: 10200322] [MGI Ref ID J:54585]
Colony Maintenance
Diet Information LabDiet® 5K52/5K67
| Pricing for USA, Canada and Mexico shipping destinations |
|
*Price(s) in US dollars ($)
Weeks of Age Price* Gender Cryorecovery Fee $1900.00
| Pricing for International shipping destinations |
|
*Price(s) in US dollars ($)
Weeks of Age Price* Gender Cryorecovery Fee $2470.00
| Standard Supply | Repository-Cryopreserved. Must Be Recovered. Please refer to pricing and supply notes for further information. |
|---|---|
| Supply Notes |
|
| Control | ||
|---|---|---|
| Wild-type from the colony | ||
| 000664 C57BL/6J | ||
| 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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Tel: 800.422.6423 or 207.288.5845
Fax: 207.288.6150
Technical Support Email Form
| phone: | 207-288-6470 |
| fax: | 207-288-6655 |
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