JAX Mouse Strain Datasheet - 025335

B6.Cg-Lamc2^tm1Uit/DcrJ

Stock No:: 025335

Cryo Recovery

Overview

This Lamc2 knock out strain may be useful in studies of epidermolysis bullosa and is a model for Herlitz Type Junctional Epidermolysis Bullosa.

Donating Investigator

Dr. Derry Roopenian, The Jackson Laboratory

Genetic overview

Genetic Background	Generation

Lamc2^tm1Uit

Allele Type	Gene Symbol	Gene Name
Targeted (Null/Knockout)	Lamc2	laminin, gamma 2

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Research Applications

Dermatology Research
Developmental Biology Research
Endocrine Deficiency Research
Internal/Organ Research

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Base Price

Starting at:

$2,595.00 Domestic price Cryo Recovery

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Details

Detailed Description

The Lamc2 gene encodes for the gamma-2 subunit of laminin 332 (laminin 5), which is an extracellular matrix glycoprotein found in cutaneous basement membranes. These mice carry a knock out mutation of Lamc2 which results in a frameshift deletion of exon 8. Mice that are homozygous for the targeted mutation die by 5 days of age. Heterozgyotes are viable and fertile. No protein gene product is detected by immunofluorescence screening of skin from homozygotes. A truncated 291bp transcript is detected by RT-PCR analysis of skin from homozygotes and heterozygotes. Onset of the progressive blistering phenotype occurs within 1 to 2 days of birth in almost all homozygous mice. Dermal-epidermal tissue separation is found in the skin and mucous membranes (oral mucosa, urinary bladder).

Development

A loxP site flanked targeting vector designed containing a NEO-TK cassette was utilized in the construction of this mutant. This selection cassette and a FRT site was inserted downstream of exon 8 of the targeted gene, and another loxP site was inserted upstream of exon 8. A second FRT site was inserted downstream of exon 9. The construct was electroporated into unspecified embryonic stem (ES) cells which were transiently transfected with a Cre recombinase vector to remove the selection cassette. ES cells that had successfully undergone Cre-mediated recombination and no longer retained the cassette or exon 8 were injected into C57BL/6J blastocysts. The resulting chimeric animals were crossed to C57BL/6J mice. The mice were then backcrossed to C57BL/6J for 13 generations. Upon arrival at The Jackson Laboratory Repository, the mice were crossed to C57BL/6J (Stock No. 000664) at least once to establish the colony.

Control Suggestions

Wild-type from the colony
000664 C57BL/6J

Additional Information

Considerations for Choosing Controls

Selected References

Meng X; Klement JF; Leperi DA; Birk DE; Sasaki T; Timpl R; Uitto J; Pulkkinen L. 2003. Targeted inactivation of murine laminin gamma2-chain gene recapitulates human junctional epidermolysis bullosa. J Invest Dermatol 121(4):720-31. PubMed: 14632187 MGI: J:85981

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Genetics

Lamc2^tm1Uit

Allele Symbol: Lamc2^tm1Uit

Allele Name	targeted mutation 1, Jouni Uitto
Allele Type	Targeted (Null/Knockout)
Allele Synonym(s)	Lamc2-
Gene Symbol and Name	Lamc2, laminin, gamma 2
Gene Synonym(s)	AA589349; B2T; BM600; CSF; EBR2; EBR2A; LAMB2T; LAMNB2; expressed sequence AA589349; nicein, 100kDa
Strain of Origin	Not Specified
Chromosome	1
Molecular Note	A floxed neomycin resistance gene was inserted in intron 8 and another loxP site was placed in intron 7. Also, frt sites were inserted on either side of exon 9. Transient cre expression in the ES cells removed exon 8 which caused a frame shift and a premature termination codon in exon 9. RT-PCR indicated mRNA of 510bp rather than 623bp. There was also a minor smaller transcript of 291bp. No protein product could be seen immunologically in skin biopsies of homozygous mice.

Disease/Phenotype

Disease Terms

Research Areas By Genotype

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

Dermatology Research
- Skin and Hair Texture Defects
Developmental Biology Research
- Internal/Organ Defects
  - skin
- Perinatal Lethality
  - Homozygous
- Skin and Hair Texture Defects
Endocrine Deficiency Research
- Skin Defects
Internal/Organ Research
- Bladder

Mammalian Phenotype Terms by Genotype

Genotype: Lamc2^tm1Uit/Lamc2^tm1Uit
involves: C57BL/6

mortality/aging

postnatal lethality, complete penetrance
- affected animals die by about day 5 postnatally

behavior/neurological phenotype

aphagia
- by day 2, milk content in stomachs begins to drop

digestive/alimentary phenotype

abnormal digestive system morphology
- separation of tissue layers in oral mucosa

renal/urinary system phenotype

abnormal kidney morphology

abnormal urinary bladder urothelium morphology
- tissue layer separations in the epithelium

integument phenotype

abnormal skin morphology
- apoptosis in basal layers of skin
- hypoplastic and rudimentary hemidesmosomes in the skin seen by transmission EM

blistering
- appears within 1-2 days of birth in 90% of mice
- mice lacking blistering are smaller and weaker than wild-type
- mostly on paws and legs

dermal-epidermal separation
- dermal-epidermal tissues separating

References

Meng X; Klement JF; Leperi DA; Birk DE; Sasaki T; Timpl R; Uitto J; Pulkkinen L. 2003. Targeted inactivation of murine laminin gamma2-chain gene recapitulates human junctional epidermolysis bullosa. J Invest Dermatol 121(4):720-31. PubMed: 14632187 MGI: J:85981

Additional - Lamc2^tm1Uit related

Adair-Kirk TL; Griffin GL; Meyer MJ; Kelley DG; Miner JH; Keene DR; Marinkovich MP; Ruppert JM; Uitto J; Senior RM. 2012. Keratinocyte-targeted expression of human laminin gamma2 rescues skin blistering and early lethality of laminin gamma2 deficient mice. PLoS One 7(9):e45546. PubMed: 23029085 MGI: J:191995
Bubier JA; Sproule TJ; Alley LM; Webb CM; Fine JD; Roopenian DC; Sundberg JP. 2010. A Mouse Model of Generalized Non-Herlitz Junctional Epidermolysis Bullosa. J Invest Dermatol 130(7):1819-28. PubMed: 20336083 MGI: J:158873
Sproule TJ; Bubier JA; Grandi FC; Sun VZ; Philip VM; McPhee CG; Adkins EB; Sundberg JP; Roopenian DC. 2014. Molecular identification of collagen 17a1 as a major genetic modifier of laminin gamma 2 mutation-induced junctional epidermolysis bullosa in mice. PLoS Genet 10(2):e1004068. PubMed: 24550734 MGI: J:211010
Wazen RM; Viegas-Costa LC; Fouillen A; Moffatt P; Adair-Kirk TL; Senior RM; Nanci A. 2016. Laminin gamma2 knockout mice rescued with the human protein exhibit enamel maturation defects. Matrix Biol 52-54:207-18. PubMed: 26956061 MGI: J:237089

Pricing & Availability

Cryo Recovery

Domestic
International

Live Mouse

Age

Genotype

Price

weeks

Cryorecovery - Pricing

Service	Genotype	Price
	Please inquire about possible genotypes.

Progeny testing is not required

The average number of mice provided from recovery of our cryopreserved strains is 10. The total number of animals provided, their gender and genotype will vary. We will fulfill your order by providing at least two pair of mice, at least one animal of each pair carrying the mutation of interest. Please inquire if larger numbers of animals with specific genotype and genders are needed. Animals typically ship between 10 and 14 weeks from the date of your order. If a second cryorecovery is needed in order to provide the minimum number of animals, animals will ship within 25 weeks.

The genotypes of 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 about possible genotypes which will be recovered for this specific strain. The Jackson Laboratory cannot guarantee the reproductive success of mice shipped to your facility. If the mice are lost after the first three days (post-arrival) or do not produce progeny at your facility, a new order and fee will be necessary.

Cryorecovery to establish a Dedicated Supply for greater quantities of mice. Mice recovered can be used to establish a dedicated colony to contractually supply you mice according to your requirements. Price by quotation.

Payment Terms and Conditions

Terms are granted by individual review and stated on the customer invoice(s) and account statement. These transactions are payable in U.S. currency within the granted terms. Payment for services, products, shipping containers, and shipping costs that are rendered are expected within the payment terms indicated on the invoice or stated by contract. Invoices and account balances in arrears of stated terms may result in The Jackson Laboratory pursuing collection activities including but not limited to outside agencies and court filings.

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.

Terms Of Use

Terms of Use

General Terms and Conditions

Questions about Terms of Use

Licensing Information

Phone: 207-288-6470

Email: tjlca@jax.org

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Donating Investigator

Genetic overview

Research Applications

Base Price

Detailed Description

Development

Control Suggestions

Additional Information

Selected References

Lamc2tm1Uit

Allele Symbol: Lamc2tm1Uit

Disease Terms

Research Areas By Genotype

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

Mammalian Phenotype Terms by Genotype

Genotype: Lamc2tm1Uit/Lamc2tm1Uitinvolves: C57BL/6

mortality/aging

behavior/neurological phenotype

digestive/alimentary phenotype

renal/urinary system phenotype

integument phenotype

References

Additional - Lamc2tm1Uit related

Genotyping Protocols

Breeding Considerations

Animal Health Reports

Production of mice from cryopreserved embryos or sperm occurs in a maximum barrier room, G200

Live Mouse

Cryorecovery - Pricing

Progeny testing is not required

Payment Terms and Conditions

The Jackson Laboratory's Genotype Promise

Terms of Use

Licensing Information

JAX® Mice, Products & Services Conditions of Use

No Warranty

Credit for PRODUCTS or SERVICES

Animal Care and Use for SERVICES

No Liability

Lamc2^tm1Uit

Allele Symbol: Lamc2^tm1Uit

Genotype: Lamc2^tm1Uit/Lamc2^tm1Uit
involves: C57BL/6

Additional - Lamc2^tm1Uit related