Overview

Also Known As:Ptprq-CAT-KO

These Ptprq knock-out mice have abnormal development of the organ of Corti and exhibit deafness.

Donating Investigator

Daniel F. Bowen-Pope, University of Washington 357470

Genetic overview

Genetic Background	Generation

Ptprq^tm2Bow

Allele Type	Gene Symbol	Gene Name
Targeted (Null/Knockout)	Ptprq	protein tyrosine phosphatase, receptor type, Q

View Genetics

Research Applications

Neurobiology Research
Sensorineural Research

View All Research Applications

Base Price

Starting at:

$2,854.50 Domestic price Cryo Recovery

View Price List

Details

Detailed Description

Mice that are homozygous for the targeted mutation are viable, fertile, normal in size and do not display any gross physical or behavioral abnormalities. No gene product (protein) is detected by immunofluorescence labeling analysis of the inner ear. Homozygotes do not respond to the 20kHz toneburst of a hearing test at 3 months of age. Although embryonic development of the inner ear is normal, newborns exhibit disorganized and defective hair cell bundles. Histological analysis reveals an absence of hair cells in the organ of Corti and some animals are missing the organ of Corti completely. Inner hair cells have missing or defective stereocilia. This mutant mouse strain may be useful in studies related to deafness.

Development

A targeting vector containing neomycin resistance and herpes simplex virus thymidine kinase genes was used to disrupt 1061 bp sequence containing the 2 exons encoding the catalytic domain and flanking sequence. The construct was electroporated into 129-derived R1 embryonic stem (ES) cells. Correctly targeted ES cells were injected into C57BL/6 blastocysts.

Control Suggestions

Wildtype mice from the colony or C57BL/6J (000664) can be used as controls.
Wild-type from the colony

Additional Information

Considerations for Choosing Controls

Selected References

Goodyear RJ; Legan PK; Wright MB; Marcotti W; Oganesian A; Coats SA; Booth CJ; Kros CJ; Seifert RA; Bowen-Pope DF; Richardson GP. 2003. A receptor-like inositol lipid phosphatase is required for the maturation of developing cochlear hair bundles. J Neurosci 23(27):9208-19PubMed: 14534255 MGI: J:85926

View All References

Genetics

Ptprq^tm2Bow

Allele Symbol: Ptprq^tm2Bow

Allele Name	targeted mutation 2, Daniel F Bowen-Pope
Allele Type	Targeted (Null/Knockout)
Allele Synonym(s)	Ptprq-CAT-KO
Gene Symbol and Name	Ptprq, protein tyrosine phosphatase, receptor type, Q
Gene Synonym(s)
Strain of Origin	129/Sv
Chromosome	10
Molecular Note	A 1061 bp fragment encompassing 2 exons, encoding a portion of the cytoplasmic domain including the catalytic site, was replaced with a neomycin selection cassette inserted by homologous recombination. While, mutant transcript lacking the targeted fragment was detected by RT-PCR analysis of cochlear RNA obtained from homozygous mutant mice, protein was undetected by immunostaining. Absence of protein was further verified using antisera to the C-terminal.
Mutations Made By	Daniel Bowen-Pope, University of Washington 357470

Disease/Phenotype

Disease Terms

Research Areas By Phenotype

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

Genotype: Ptprq^tm2Bow related

Neurobiology Research
- Hearing Defects
Sensorineural Research
- Hearing Defects

Mammalian Phenotype Terms by Genotype

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

Genotype: Ptprq^tm2Bow/Ptprq^tm2Bow
involves: 129 * C57BL/6

behavior/neurological phenotype

absent pinna reflex
- at 3 months of age, homozygotes fail to respond with a Preyer's reflex to a 20 kHz tone burst
- Normal - however, no overt behavioral vestibular phenotype is observed
- however, no overt, behavioral vestibular phenotype is observed

nervous system phenotype

abnormal cochlear hair cell stereociliary bundle morphology
- by P8, defects in hair-bundle structure become more pronounced and are readily observed on both IHCs and OHCs
- defects in hair-bundle structure are first apparent in IHCs at P1

abnormal hair cell mechanoelectric transduction
- basal-coil OHCs obtained from homozygotes (aged P5-P7) show a reduction in mechanoelectrical transducer current amplitudes relative to heterozygotes, most likely due to the loss of stereocilia
- Normal - however, the shape of transducer currents, fast, submillisecond adaptation and slow adaptation at both the onset of excitatory and the offset of inhibitory stimuli, and the resting transducer current at hyperpolarized and depolarized potentials, remain normal
- Normal - rmal

abnormal inner hair cell stereociliary bundle morphology
- at P1, homozygotes display varying degrees of disorganization in the hair-bundle structure of IHCs in the basal-most regions of the cochlea, not evident at E18.5

abnormal orientation of inner hair cell stereociliary bundles
- remaining IHC stereocilia are often misaligned

abnormal outer hair cell stereociliary bundle morphology
- by P8, homozygotes display disorganization in the hair-bundle structure of OHCs in the basal-most regions of the cochlea
- mutant hair bundles fail to develop the typical broader, flatter, and wider-angled appearance of wild-type hair bundles in the basal end of the cochlea
- mutant OHC hair bundles are smaller, exhibit a less distinctive V-shaped form and are more U-shaped

abnormal vestibular hair cell stereociliary bundle morphology
- close apposition of the stereocilia is noted in ruthenium red-stained preparations; however, the spacing of stereocilia appears normal in the absence of ruthenium red

absent vestibular hair bundle shaft connectors
- at P21, homozygotes exhibit complete absence of shaft connectors (defined as dense particles plus stalks), from the extrastriolar hair bundles of the utricular macula
- Normal - however, the stereocilia forming the hair bundle are not splayed

cochlear hair cell degeneration
- at 3 months of age, homozygotes show absence of hair cells in the basal, high-frequency end while the apical, low-frequency end of the cochlea appears unaffected
- hair cells are still present in the organ of Corti until at least P22; however, complete loss of hair cells is noted between P21 and P80
- Normal - in contrast, no hair cell loss is observed in the vestibular organs

decreased inner hair cell stereocilia number
- many IHC stereocilia are absent

fused inner hair cell stereocilia
- remaining IHC stereocilia are often fused

short outer hair cell stereocilia
- OHC stereocilia are shorter than normal; however, stereocilial fusion is rarely observed

hearing/vestibular/ear phenotype

abnormal cochlear hair cell stereociliary bundle morphology
- by P8, defects in hair-bundle structure become more pronounced and are readily observed on both IHCs and OHCs
- defects in hair-bundle structure are first apparent in IHCs at P1

abnormal hair cell mechanoelectric transduction
- basal-coil OHCs obtained from homozygotes (aged P5-P7) show a reduction in mechanoelectrical transducer current amplitudes relative to heterozygotes, most likely due to the loss of stereocilia
- Normal - however, the shape of transducer currents, fast, submillisecond adaptation and slow adaptation at both the onset of excitatory and the offset of inhibitory stimuli, and the resting transducer current at hyperpolarized and depolarized potentials, remain normal
- Normal - rmal

abnormal inner hair cell stereociliary bundle morphology
- at P1, homozygotes display varying degrees of disorganization in the hair-bundle structure of IHCs in the basal-most regions of the cochlea, not evident at E18.5

abnormal orientation of inner hair cell stereociliary bundles
- remaining IHC stereocilia are often misaligned

abnormal outer hair cell stereociliary bundle morphology
- by P8, homozygotes display disorganization in the hair-bundle structure of OHCs in the basal-most regions of the cochlea
- mutant hair bundles fail to develop the typical broader, flatter, and wider-angled appearance of wild-type hair bundles in the basal end of the cochlea
- mutant OHC hair bundles are smaller, exhibit a less distinctive V-shaped form and are more U-shaped

abnormal vestibular hair cell stereociliary bundle morphology
- close apposition of the stereocilia is noted in ruthenium red-stained preparations; however, the spacing of stereocilia appears normal in the absence of ruthenium red

absent vestibular hair bundle shaft connectors
- at P21, homozygotes exhibit complete absence of shaft connectors (defined as dense particles plus stalks), from the extrastriolar hair bundles of the utricular macula
- Normal - however, the stereocilia forming the hair bundle are not splayed

cochlear hair cell degeneration
- at 3 months of age, homozygotes show absence of hair cells in the basal, high-frequency end while the apical, low-frequency end of the cochlea appears unaffected
- hair cells are still present in the organ of Corti until at least P22; however, complete loss of hair cells is noted between P21 and P80
- Normal - in contrast, no hair cell loss is observed in the vestibular organs

deafness

decreased inner hair cell stereocilia number
- many IHC stereocilia are absent

fused inner hair cell stereocilia
- remaining IHC stereocilia are often fused

organ of Corti degeneration
- between P21 and P80, some homozygotes exhibit a complete loss of the organ of Corti

short outer hair cell stereocilia
- OHC stereocilia are shorter than normal; however, stereocilial fusion is rarely observed

References

Goodyear RJ; Legan PK; Wright MB; Marcotti W; Oganesian A; Coats SA; Booth CJ; Kros CJ; Seifert RA; Bowen-Pope DF; Richardson GP. 2003. A receptor-like inositol lipid phosphatase is required for the maturation of developing cochlear hair bundles. J Neurosci 23(27):9208-19PubMed: 14534255 MGI: J:85926

Additional References

Wright MB; Hugo C; Seifert R; Disteche CM; Bowen-Pope DF. 1998. Proliferating and migrating mesangial cells responding to injury express a novel receptor protein-tyrosine phosphatase in experimental mesangial proliferative glomerulonephritis. J Biol Chem 273(37):23929-37PubMed: 9727007 MGI: J:49745

Additional - Ptprq^tm2Bow related

Technical Support

CONTACT TECHNICAL SUPPORT

Genotyping Protocols
- Standard PCR:Ptprq
- Genotyping resources and troubleshooting
Breeding Considerations

This strain originated on a B6;129 background, was backcrossed for 6 generations on the C57BL/6J background.
- Additional Breeding and Husbandry Support
Citation
When using the Ptprq-CAT-KO mouse strain in a publication, please cite the originating article(s) and include JAX stock #004833 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/Product	Description	Price
	Heterozygous or Wild-type for Ptprq<tm2Bow>

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. We do not guarantee breeding performance and therefore suggest that investigators order more than one breeding pair to avoid delays in their research.

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.

Also Known As:Ptprq-CAT-KO

Donating Investigator

Genetic overview

Research Applications

Base Price

Detailed Description

Development

Control Suggestions

Additional Information

Selected References

Ptprqtm2Bow

Allele Symbol: Ptprqtm2Bow

Disease Terms

Research Areas By Phenotype

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

Genotype: Ptprqtm2Bow related

Mammalian Phenotype Terms by Genotype

Genotype: Ptprqtm2Bow/Ptprqtm2Bowinvolves: 129 * C57BL/6

behavior/neurological phenotype

nervous system phenotype

hearing/vestibular/ear phenotype

References

Additional References

Additional - Ptprqtm2Bow 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

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

Ptprq^tm2Bow

Allele Symbol: Ptprq^tm2Bow

Genotype: Ptprq^tm2Bow related

Genotype: Ptprq^tm2Bow/Ptprq^tm2Bow
involves: 129 * C57BL/6

Additional - Ptprq^tm2Bow related