Overview

This spontaneous frameshift, predicted to modify only the last of the four zinc finger domains in ZFP191, is valuable in the study of the transcriptional regulation in oligodendrocytes that is essential for myelination of axons in the central nervous system.

Genetic overview

Genetic Background	Generation

Zfp24^hmcns-2J

Allele Type	Gene Symbol	Gene Name
Spontaneous	Zfp24	zinc finger protein 24

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Research Applications

Cell Biology Research
Neurobiology Research

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Base Price

Starting at:

$2,854.50 Domestic price Cryo Recovery

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Details

Detailed Description

Mice homozygous for the Zfp191^hmcns-2J mutation have very little myelin in the central nervous system when assessed at four weeks of age, but no myelin deficiency in the peripheral nervous system. Homozygotes can be identified as early as fourteen days of age by a whole body tremor that is most pronounced when the mouse is moving. Both males and females have tonic seizures. The severity of the tremors becomes more pronounced as they age and they do not survive beyond four to five weeks of age. Because of this, this strain must be maintained using heterozygous breeders. A hearing deficit, which varies in expressivity, has also been attributed to this mutation in this strain.

Development

A spontaneous, dominant mutation causing head tossing arose in the strain CByJ.129P2(B6)-Cd4^tm1Mak/J and this mutant subline was maintained by continuous backcross to BALB/cByJ. This removed the Cd4^tm1Mak mutation by generation N2 and at generation N7 a new mutation arose spontaneously that was subsequently characterized as the Zfp191^hmcns-2J mutation. Because this recessive Zfp191^hmcns-2J mutation causes death before breeding age this mutant subline was maintained by sibling intercrossing heterozygotes, which were proven by progeny testing. The dominant head tossing mutation was easily bred out by phenotypic selection. The CByJ(Cg)-Zfp191^hmcns-2J/GrsrJ strain reached generation +F3 in 2013.

Control Suggestions

+/? from the colony
001026 BALB/cByJ

Additional Information

Considerations for Choosing Controls

Selected References

Dionne L; Bronson RT; Berry ML; Bergstrom DE; Donahue LR. 2013. A spontaneous Zfp191 mouse mutation that alters 18 carboxyl terminal amino acids MGI Direct Data SubmissionMGI: J:202595

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Genetics

Zfp24^hmcns-2J

Allele Symbol: Zfp24^hmcns-2J

Allele Name	hypomyelinated central nervous system 2 Jackson
Allele Type	Spontaneous
Allele Synonym(s)	hypomyelinated central nervous system 2 Jackson; Zfp24^hmcns-2J
Gene Symbol and Name	Zfp24, zinc finger protein 24
Gene Synonym(s)	AI642085; ZSCAN3; 3526401F17Rik; 5033419P20Rik; Znf24; RIKEN cDNA 5033419P20 gene; RIKEN cDNA 3526401F17 gene; AI480505; KOX17; ZNF191; Zfp191; zinc finger protein 191; expressed sequence AI480505; expressed sequence AI642085; expressed sequence AL024422; 5033419P20Rik; RSG-A; 3526401F17Rik; ZF-12; AL024422; Zfp191
Strain of Origin	BALB/cByJ
Chromosome	18
Molecular Note	This spontaneous two base pair deletion in Chromosome 18 postitions 24,014,204 and 24,014,205 (GRCm38), which falls within exon 4, causes a frameshift that results in the substitution of 20 new amino acids in place of the last 18 amino acids of this 368 residue protein, thereby altering the last of four zinc finger domains.

Disease/Phenotype

Disease Terms

Research Areas By Genotype

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

Genotype: Zfp24^hmcns-2J related

Cell Biology Research
- Transcriptional Regulation
Neurobiology Research
- Myelination Defects
- Tremor Defects

Mammalian Phenotype Terms by Genotype

The following phenotype relates to a compound genotype created using this strain

Genotype: Zfp24^hmcns-2J/Zfp24^hmcns-2J
CByJ(Cg)-Zfp24/GrsrJ

behavior/neurological phenotype

tonic seizures
- both female and male homozygotes have tonic seizures

tremors
- evident by 14 days of age, most apparent when moving, and increasing in severity until death before four to five weeks of age

hearing/vestibular/ear phenotype

increased or absent threshold for auditory brainstem response
- at four weeks of age 5 out of 7 homozygotes show elevated thresholds indicative of hearing loss

mammalian phenotype

vision/eye phenotype
- Normal - the eyes of four homozygotes at three weeks of age appeared normal by ophthalmascopy

mortality/aging

premature death
- all homozygotes die by four to five weeks of age

nervous system phenotype

abnormal myelination

decreased myelin sheath thickness
- at four weeks of age very little myelin is found in the central nervous system, but there is normal myelination in the peripheral nervous system

tonic seizures
- both female and male homozygotes have tonic seizures

References

Dionne L; Bronson RT; Berry ML; Bergstrom DE; Donahue LR. 2013. A spontaneous Zfp191 mouse mutation that alters 18 carboxyl terminal amino acids MGI Direct Data SubmissionMGI: J:202595

Additional - Zfp24^hmcns-2J related

Technical Support

Contact Technical Support

Genotyping Protocols
- End Point Analysis:Zfp191^hmcns-2J
- Genotyping resources and troubleshooting
Mating System
- Progeny Tested
- intercross of progeny tested heterozygote
Citation
When using the CByJ(Cg)-Zfp24^hmcns-2J/GrsrJ mouse strain in a publication, please cite the originating article(s) and include JAX stock #022138 in your Materials and Methods section.

Animal Health Reports

Facility Barrier Level Descriptions

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

Pricing & Availability

Cryo Recovery

Domestic
International

Live Mouse

Age

Genotype

Price

weeks

Cryorecovery - Pricing

Service	Genotype	Price
	Heterozygous or wildtype for Zfp191<hmcns-2J>

We will fulfill your order by providing at least two carriers for each strain ordered. The total number, sex, and genotypes provided will vary, although typically 8 or more animals are provided. Please check genotypes which will be recovered. While the genotypes of all animals produced will be communicated to you prior to scheduling shipment, the genotypes of animals provided may not reflect the mating scheme and genotypes described in the strain description. Animals are typically ready to ship in 11-14 weeks. If a second recovery is required to produce the minimum number of animals, then delivery time would increase to approximately 25 weeks. If we fail to produce animals of the correct genotype, you will not be charged. We 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.

Related Products and Services

Frozen Mouse Embryo	CByJ(Cg)-Zfp24<hmcns-2J>/GrsrJ	$2595.00
Frozen Mouse Embryo	CByJ(Cg)-Zfp24<hmcns-2J>/GrsrJ	$2595.00
Frozen Mouse Embryo	CByJ(Cg)-Zfp24<hmcns-2J>/GrsrJ	$3373.50
Frozen Mouse Embryo	CByJ(Cg)-Zfp24<hmcns-2J>/GrsrJ	$3373.50

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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.

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Genetic overview

Research Applications

Base Price

Detailed Description

Development

Control Suggestions

Additional Information

Selected References

Zfp24hmcns-2J

Allele Symbol: Zfp24hmcns-2J

Disease Terms

Research Areas By Genotype

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

Genotype: Zfp24hmcns-2J related

Mammalian Phenotype Terms by Genotype

Genotype: Zfp24hmcns-2J/Zfp24hmcns-2JCByJ(Cg)-Zfp24/GrsrJ

behavior/neurological phenotype

hearing/vestibular/ear phenotype

mammalian phenotype

mortality/aging

nervous system phenotype

References

Additional - Zfp24hmcns-2J related

Genotyping Protocols

Mating System

Citation

Animal Health Reports

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

Live Mouse

Cryorecovery - Pricing

Related Products and Services

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

Zfp24^hmcns-2J

Allele Symbol: Zfp24^hmcns-2J

Genotype: Zfp24^hmcns-2J related

Genotype: Zfp24^hmcns-2J/Zfp24^hmcns-2J
CByJ(Cg)-Zfp24/GrsrJ

Additional - Zfp24^hmcns-2J related