Overview

Also Known As:pewter

These mice carry a spontaneous mutation at the Atp7a locus characterized by a light grey coat in males and mottled coat in females due to deficiencies in a number of copper-requiring enzymes attributed to an intracellular copper transport defect.

Genetic overview

Genetic Background	Generation

Atp7a^Mo-pew

Allele Type	Gene Symbol	Gene Name
Spontaneous	Atp7a	ATPase, Cu++ transporting, alpha polypeptide

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Research Applications

Metabolism Research
Dermatology Research
Developmental Biology Research
Neurobiology Research
Mouse/Human Gene Homologs

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Base Price

Starting at:

$2,854.50 Domestic price Cryo Recovery

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Details

Important Note

This strain is homozygous for the retinal degeneration allele Pde6b^rd1.

Detailed Description

The Atp7 genes encode Cu2⁺ ATPases. Atp7a is the mouse ortholog of the human ATP7A gene, which is mutated in children with Menke's syndrome. The range of defects in both Menke's syndrome and in mice having mutations of Atp7a--the mottled series of mutants--are due to deficiencies in a number of copper-requiring enzymes, in turn attributed to an intracellular copper transport defect. Thus, it is likely that the Atp7a gene product functions as a copper transport protein. (For brief review and references, see Levinson et al., 1994.)

Atp7a^Mo-pew, the mottled-pewter mutation, has the mildest effect of the known mutations at this locus. The sole phenotype noted in hemizygous males and homozygous females is their pale, silvery gray coat color. The coats of affected mice of both sexes are initially agouti; males become pewter by about 4 weeks and females at 5-6 weeks of age. Heterozygous females have normal, agouti coats throughout life. (Fox and Eicher 1978; Reynolds 2000). Both sexes are fertile (Fox and Eicher 1978).

At the molecular level, the Atp7a^Mo-pew mutation consists of a single point mutation at nucleotide 3074, in exon 15. The G-->A transition causes the substitution of a threonine for an alanine. The affected amino acid occurs in a seven amino acid sequence that is highly conserved among the twenty-one known or presumed copper-transporting ATPases. This sequence comprises the transduction domain in the sixth transmembrane segment of the protein and is believed to be responsible for its copper binding and transport functions (Levinson et al., 1997.)

Development

Atp7a^Mo-pew occurred spontaneously in 1976 on the CBA/J inbred strain at The Jackson Laboratory.

Control Suggestions

Heterozygote female from the colony
Wild-type male from the colony

Additional Information

Considerations for Choosing Controls

Genetics

Atp7a^Mo-pew

Allele Symbol: Atp7a^Mo-pew

Allele Name	pewter
Allele Type	Spontaneous
Allele Synonym(s)	Pew
Gene Symbol and Name	Atp7a, ATPase, Cu++ transporting, alpha polypeptide
Gene Synonym(s)
Strain of Origin	CBA/J
Chromosome	X
Molecular Note	This allele comprises a G-to-A substitution at coding nucleotide 2995 in exon 15 creating a missense mutation that alters codon 999 from an alanine to a threonine in a conserved region of the transduction domain of the encoded protein.

Disease/Phenotype

Disease Terms

Research Areas By Phenotype

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

Genotype: Atp7a^Mo-pew related

Metabolism Research
Dermatology Research
- Color and White Spotting Defects
Developmental Biology Research
- Defects in Extracellular Matrix Molecules
Neurobiology Research
- Metabolic Defects
Mouse/Human Gene Homologs
- Menkes syndrome

Mammalian Phenotype Terms by Genotype

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

Genotype: Atp7a^Mo-pew/Atp7a⁺
CBA/J-Atp7a

integument phenotype

abnormal coat/hair pigmentation

irregular coat pigmentation
- mice have a variable mosaic gray and wild-type agouti black coat due to X-inactivation

mottled coat
- mice have a mosaic agouti gray and agouti black coat

pigmentation phenotype

abnormal coat/hair pigmentation

irregular coat pigmentation
- mice have a variable mosaic gray and wild-type agouti black coat due to X-inactivation

mottled coat
- mice have a mosaic agouti gray and agouti black coat

Genotype: Atp7a^Mo-pew/Y
CBA/J-Atp7a

integument phenotype

abnormal coat/hair pigmentation
- males have a pale silvery coat color

diluted coat color
- males are light gray

mammalian phenotype

mortality/aging
- Normal - mice hemizygous for this allele are viable; males hemizygous for any of the non-pewter alleles die before weaning

reproductive system phenotype
- Normal - mice hemizygous for this allele are fertile; males hemizygous for any of the non-pewter alleles die before sexual maturity

pigmentation phenotype

abnormal coat/hair pigmentation
- males have a pale silvery coat color

diluted coat color
- males are light gray

References

Additional References

Levinson B; Vulpe C; Elder B; Martin C; Verley F; Packman S; Gitschier J. 1994. The mottled gene is the mouse homologue of the Menkes disease gene [see comments] Nat Genet 6(4):369-73PubMed: 8054976 MGI: J:17492

Additional - Atp7a^Mo-pew related

Technical Support

CONTACT TECHNICAL SUPPORT

Genotyping Protocols
- Genotyping resources and troubleshooting
Appearance
- light gray
  Related Genotype: A/A Atp7a^Mo-pew/Y male or A/A Atp7a^Mo-pew/Atp7a^Mo-pew female
  
  agouti
  Related Genotype: A/A +/Y male, A/A Atp7a^Mo-pew/+ female
Citation
When using the pewter mouse strain in a publication, please cite the originating article(s) and include JAX stock #000813 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
	X linked - Heterozygous Females and Wild-type Males for Mo<pew>, 1 pair minimum

Related Products and Services

Frozen Mouse Embryo	CBA/J-Atp7a<Mo-pew>/J Frozen Embryos	$2595.00
Frozen Mouse Embryo	CBA/J-Atp7a<Mo-pew>/J Frozen Embryos	$2595.00
Frozen Mouse Embryo	CBA/J-Atp7a<Mo-pew>/J Frozen Embryos	$3373.50
Frozen Mouse Embryo	CBA/J-Atp7a<Mo-pew>/J Frozen Embryos	$3373.50

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.

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General Terms and Conditions

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Licensing Information

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Also Known As:pewter

Genetic overview

Research Applications

Base Price

Important Note

Detailed Description

Development

Control Suggestions

Additional Information

Atp7aMo-pew

Allele Symbol: Atp7aMo-pew

Disease Terms

Research Areas By Phenotype

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

Genotype: Atp7aMo-pew related

Mammalian Phenotype Terms by Genotype

Genotype: Atp7aMo-pew/Atp7a+CBA/J-Atp7a

integument phenotype

pigmentation phenotype

Genotype: Atp7aMo-pew/YCBA/J-Atp7a

integument phenotype

mammalian phenotype

pigmentation phenotype

References

Additional References

Additional - Atp7aMo-pew related

Genotyping Protocols

Appearance

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

Atp7a^Mo-pew

Allele Symbol: Atp7a^Mo-pew

Genotype: Atp7a^Mo-pew related

Genotype: Atp7a^Mo-pew/Atp7a⁺
CBA/J-Atp7a

Genotype: Atp7a^Mo-pew/Y
CBA/J-Atp7a

Additional - Atp7a^Mo-pew related