JAX Mouse Strain Datasheet - 000974

C57BL/6J-Lgi4^clp/J

Stock No:: 000974

Cryo Recovery

Overview

Genetic overview

Genetic Background	Generation

Lgi4^clp

Allele Type	Gene Symbol	Gene Name
Spontaneous	Lgi4	leucine-rich repeat LGI family, member 4

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Research Applications

Neurobiology Research

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Base Price

Starting at:

$2,595.00 Domestic price Cryo Recovery

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Details

Development

The claw paw mutation arose spontaneously in the B6.Cg-Lep^ob/J strain at The Jackson Laboratory in1977. The obese mutation was bred out by backcross-intercross to C57BL/6J and this mutant subline was subsequently maintained by sibling intercrossing either homozygote x heterozygote or heterozygote x heterozygote. In 1984 embryos were generated for cryopreservation from heterozygous males and C57BL/6J females.

Control Suggestions

Untyped from the colony
000664 C57BL/6J

Additional Information

Considerations for Choosing Controls

Genetics

Lgi4^clp

Allele Symbol: Lgi4^clp

Allele Name	claw paw
Allele Type	Spontaneous
Allele Synonym(s)
Gene Symbol and Name	Lgi4, leucine-rich repeat LGI family, member 4
Gene Synonym(s)	AMCNMY; LGIL3; claw paw; clp; clp
Strain of Origin	B6.Cg-Lep^ob/J
Chromosome	7
General Note	This mutation arose at The Jackson Laboratory in 1977. The pathology of homozygous mutant mice differs from other myelin deficient mutants in that there is no obvious increase in Schwann cells and that the axons that persist in promyelin fibers are smaller (J:11392).
Molecular Note	A CTCT repeat located six base pairs 5' to exon 4 is replaced with a 225-bp repetitive sequence which is 100% homologous to a repeat element located 797 bp upstream. This interferes with the third intron 3' splice site resulting in exon3 spliced to exon 5. The resulting protein, lacking exon 4, is not secreted as is the wild-type allele.

Disease/Phenotype

Disease Terms

Research Areas By Genotype

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

Genotype: Lgi4^clp related

Neurobiology Research
- Myelination Defects

Mammalian Phenotype Terms by Genotype

Genotype: Lgi4^clp/Lgi4^clp
involves: C57BL/6J

nervous system phenotype

demyelination
- in the sciatic nerve at P12

Genotype: Lgi4^clp/Lgi4^clp
C57BL/6J-Lgi4^clp/J

mortality/aging

postnatal lethality, incomplete penetrance
- homozygotes with severe forelimb abnormalities often die within 2 to 3 days after birth due to difficulties in positioning themselves for suckling and thus do not get enough nourishment, however if they do survive the first few weeks, their condition usually begins to improve and some have a relatively normal lifespanlly begins to improve and some have a relatively normal lifespan

growth/size/body region phenotype

decreased body size
- adults with forelimb abnormalities are almost always smaller than controls

nervous system phenotype

abnormal axon morphology
- the axons that are enclosed by promyelin fibers have smaller axon calibers than those of myelinated fibers

abnormal myelin sheath morphology
- in some mutants, the ratio of myelin sheath thickness to axon caliber is reduced
- variable degree of generalized hypomyelination, with peripheral myelin sheaths thinner relative to axonal size than in controls

abnormal myelination
- all homozygotes have delayed and abnormal myelination of the peripheral nervous system but not the central nervous system
- delay in myelination and a persistence of some axons and Schwann cells in the promyelin configuration throughout life
- failure of myelination in some fibers leads to persistent promyelin fibers with small axons, especially in muscles that normally have small axons
- neuron-Schwann cell cocultures from E13 dissociated dorsal root ganglia do not myelinate

abnormal nervous system morphology
- all peripheral nerves are hypotrophic
- exhibit a variable increase in peripheral nerve fasciculation and an occasional mild increase in endoneural connective tissue, however no differences in the number of Schwann cells or any evidence of denervation atrophy

abnormal sciatic nerve morphology
- hypertrophy of the sciatic nerve

limbs/digits/tail phenotype

abnormal digit morphology
- fingers are flexed

abnormal forelimb morphology
- flexed forelimbs (fingers, wrists, shoulders), elbows rotated inwards
- forelimbs with abnormal postures are held flexed at one or more of the shoulder, wrist or digital joints but extended at the elbow, resulting in the forelimbs being pointed toward the hindlimbs

abnormal hindlimb morphology
- hindlimbs are more subtly affected than forelimbs, with the most common abnormality a mild, usually symmetrical, weakness of hindpaw dorsiflexion

abnormal limb morphology
- abnormally flexed joints can be extended early in life but fixed contractures often develop with age
- exhibit an arthrogryposis-like phenotype affecting the forelimbs, and in severe cases, also the hindlimbs

decreased diameter of radius
- radii are thinner and somewhat straighter than in controls, however gross bony landmarks and sections through bones and joints appear normal

decreased diameter of ulna
- ulnae are thinner and somewhat straighter than in controls

behavior/neurological phenotype

abnormal locomotor behavior
- develop a more pronounced waddle as grow older
- homozygotes with bilateral forelimb abnormalities have a difficult time from a very young age in moving around the cage and slither around the cage on the undersurface of their muzzles

abnormal maternal nurturing
- maternal abilities are rarely good in females with severe forelimb abnormalities

abnormal posture
- abnormal forelimb joint postures seen within 1-2 days after birth
- postural deformity (waiter's tip posture) of one or both forelimbs in which the fingers are flexed and the wrist and shoulder may also be flexed and the elbow rotated inward

abnormal sexual interaction
- males breed poorly, however females are able to breed successfully

impaired righting response
- unable to extend an affected forelimb above the head as part of a righting response when turned from its back onto one side

skeleton phenotype

abnormal long bone morphology

decreased diameter of radius
- radii are thinner and somewhat straighter than in controls, however gross bony landmarks and sections through bones and joints appear normal

decreased diameter of ulna
- ulnae are thinner and somewhat straighter than in controls

muscle phenotype

muscle phenotype
- do not exhibit any histological abnormalities in skeletal muscle or any reductions in large limb muscle mass

The following phenotype information is associated with a similar, but not exact match to this JAX^® Mice strain

Genotype: Lgi4^clp/Lgi4^clp
involves: C57BL/6J * FVB/N

nervous system phenotype

abnormal myelination
- cultures of primary sensory neurons fail to myelinate

References

Additional References

Henry EW; Eicher EM; Sidman RL. 1991. The mouse mutation claw paw: forelimb deformity and delayed myelination throughout the peripheral nervous system. J Hered 82(4):287-94. PubMed: 1652607 MGI: J:11392

Additional - Lgi4^clp related

Bermingham JR Jr; Shearin H; Pennington J; O'Moore J; Jaegle M; Driegen S; van Zon A; Darbas A; Ozkaynak E; Ryu EJ; Milbrandt J; Meijer D. 2006. The claw paw mutation reveals a role for Lgi4 in peripheral nerve development. Nat Neurosci 9(1):76-84. PubMed: 16341215 MGI: J:105260
Bermingham JR Jr; Shumas S; Whisenhunt T; Sirkowski EE; O'Connell S; Scherer SS; Rosenfeld MG. 2002. Identification of genes that are downregulated in the absence of the POU domain transcription factor pou3f1 (Oct-6, Tst-1, SCIP) in sciatic nerve. J Neurosci 22(23):10217-31. PubMed: 12451123 MGI: J:80437
Darbas A; Jaegle M; Walbeehm E; van den Burg H; Driegen S; Broos L; Uyl M; Visser P; Grosveld F; Meijer D. 2004. Cell autonomy of the mouse claw paw mutation. Dev Biol 272(2):470-82. PubMed: 15282162 MGI: J:92324
Henry E; Sidman R. 1987. Claw paw, a new mutation affecting peripheral nerves Mouse News Lett 78:46. MGI: J:24743
Henry EW; Eicher EM; Sidman RL. 1991. The mouse mutation claw paw: forelimb deformity and delayed myelination throughout the peripheral nervous system. J Hered 82(4):287-94. PubMed: 1652607 MGI: J:11392
Kegel L; Jaegle M; Driegen S; Aunin E; Leslie K; Fukata Y; Watanabe M; Fukata M; Meijer D. 2014. Functional phylogenetic analysis of LGI proteins identifies an interaction motif crucial for myelination. Development 141(8):1749-56. PubMed: 24715463 MGI: J:208847
Koszowski AG; Owens GC; Levinson SR. 1998. The effect of the mouse mutation claw paw on myelination and nodal frequency in sciatic nerves. J Neurosci 18(15):5859-68. PubMed: 9671673 MGI: J:49329
Niemann S; Sidman RL; Nave KA. 1998. Evidence against altered forms of MAG in the dysmyelinated mouse mutant claw paw. Mamm Genome 9(11):903-4. PubMed: 9799842 MGI: J:50579
Ozkaynak E; Abello G; Jaegle M; van Berge L; Hamer D; Kegel L; Driegen S; Sagane K; Bermingham JR Jr; Meijer D. 2010. Adam22 is a major neuronal receptor for Lgi4-mediated Schwann cell signaling. J Neurosci 30(10):3857-64. PubMed: 20220021 MGI: J:158973

Pricing & Availability

Cryo Recovery

Domestic
International

Live Mouse

Age

Genotype

Price

weeks

Cryorecovery - Pricing

Service	Genotype	Price
	Heterozygous or Wild-type for Lgi4<clp>

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: TechTran@jax.org

JAX® Mice, Products & Services Conditions of Use

"MICE" means mouse strains, their progeny derived by inbreeding or crossbreeding, unmodified derivatives from mouse strains or their progeny supplied by The Jackson Laboratory ("JACKSON"). "PRODUCT(S)" means biological materials supplied by JACKSON, and their derivatives. "SERVICES" means projects conducted by JACKSON for other parties that may include but are not limited to the use of MICE or PRODUCTS. "RECIPIENT" means each recipient of MICE, PRODUCTS, or SERVICES provided by JACKSON including each institution, its employees and other researchers under its control. MICE or PRODUCTS shall not be: (i) used for any purpose other than internal research, (ii) sold or otherwise provided to any third party for any use, or (iii) provided to any agent or other third party to provide breeding or other services. Acceptance of MICE, PRODUCTS or SERVICES from JACKSON shall be deemed as agreement by RECIPIENT to these conditions, and departure from these conditions requires JACKSON’s prior written authorization.

No Warranty

MICE, PRODUCTS AND SERVICES ARE PROVIDED "AS IS". JACKSON EXTENDS NO WARRANTIES OF ANY KIND, EITHER EXPRESS, IMPLIED, OR STATUTORY, WITH RESPECT TO MICE, PRODUCTS OR SERVICES, INCLUDING ANY IMPLIED WARRANTY OF MERCHANTABILITY OR FITNESS FOR A PARTICULAR PURPOSE, OR ANY WARRANTY OF NON-INFRINGEMENT OF ANY PATENT, TRADEMARK, OR OTHER INTELLECTUAL PROPERTY RIGHTS.

Credit for PRODUCTS or SERVICES

In case of dissatisfaction for a valid reason and claimed in writing by a purchaser within ninety (90) days of receipt of, PRODUCTS or SERVICES, JACKSON will, at its option, provide credit or replacement for the PRODUCT received or the SERVICES provided; JACKSON makes no other representations and this shall be the exclusive remedy of the purchaser. Please note specific policy for live mice.

Animal Care and Use for SERVICES

Consistent with the requirement for a written understanding regarding animal care and use, the JACKSON Animal Care and Use Committee will review the animal care and use protocol(s) associated with any SERVICES to be performed at JACKSON, and JACKSON shall have ultimate responsibility and authority for the care of animals while on site or in JACKSON custody.

No Liability

In no event shall JACKSON, its trustees, directors, officers, employees, and affiliates be liable for any causes of action or damages, including any direct, indirect, special, or consequential damages, arising out of the provision of MICE, PRODUCTS, or SERVICES, including economic damage or injury to property and lost profits, and including any damage arising from acts or negligence on the part of JACKSON, its agents or employees. Unless prohibited by law, in purchasing or receiving MICE, PRODUCTS, or SERVICES from JACKSON, purchaser or recipient, or any party claiming by or through them, expressly releases and discharges JACKSON from all such causes of action or damages, and further agrees to defend and indemnify JACKSON from any costs or damages arising out of any third party claims.

MICE, PRODUCTS or SERVICES are to be used in a safe manner and in accordance with all applicable governmental rules and regulations.

The foregoing represents the General Terms and Conditions applicable to JACKSON’s MICE, PRODUCTS or SERVICES. In addition, special terms and conditions of sale of certain MICE, PRODUCTS, or SERVICES may be set forth separately in JACKSON web pages, catalogs, price lists, contracts, and/or other documents, and these special terms and conditions shall also govern the sale of these MICE, PRODUCTS and SERVICES by JACKSON, and by its licensees and distributors.

Acceptance of delivery of MICE, PRODUCTS or SERVICES shall be deemed agreement to these terms and conditions. No purchase order or other document transmitted by purchaser or recipient that may modify the terms and conditions hereof, shall be in any way binding on JACKSON, and instead the terms and conditions set forth herein, including any special terms and conditions set forth separately, shall govern the sale of MICE, PRODUCTS or SERVICES by JACKSON.

Genetic overview

Research Applications

Base Price

Development

Control Suggestions

Additional Information

Lgi4clp

Allele Symbol: Lgi4clp

Disease Terms

Research Areas By Genotype

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

Genotype: Lgi4clp related

Mammalian Phenotype Terms by Genotype

Genotype: Lgi4clp/Lgi4clpinvolves: C57BL/6J

nervous system phenotype

Genotype: Lgi4clp/Lgi4clpC57BL/6J-Lgi4clp/J

mortality/aging

growth/size/body region phenotype

nervous system phenotype

limbs/digits/tail phenotype

behavior/neurological phenotype

skeleton phenotype

muscle phenotype

Genotype: Lgi4clp/Lgi4clpinvolves: C57BL/6J * FVB/N

nervous system phenotype

References

Additional References

Additional - Lgi4clp related

Genotyping Protocols

Breeding Considerations

Citation

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

Lgi4^clp

Allele Symbol: Lgi4^clp

Genotype: Lgi4^clp related

Genotype: Lgi4^clp/Lgi4^clp
involves: C57BL/6J

Genotype: Lgi4^clp/Lgi4^clp
C57BL/6J-Lgi4^clp/J

Genotype: Lgi4^clp/Lgi4^clp
involves: C57BL/6J * FVB/N

Additional - Lgi4^clp related