JAX Mouse Strain Datasheet - 017485

FVB-Tg(HTT*)1Xwy/J

Stock No:: 017485 |; BAC SA

Cryo Recovery

Overview

Also Known As: BAC SA

BAC SA transgenic mice express a mutant human HTT (huntingtin) gene that produces a protein that is phosphoresistant at the NT17 domain and has a 97 polyQ repeat expansion.

Donating Investigator

X. William Yang, University of California Los Angeles

Genetic overview

Genetic Background	Generation

Tg(HTT*)1Xwy

Allele Type
Transgenic (Humanized sequence, Inserted expressed sequence)

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Research Applications

Neurobiology Research
Developmental Biology Research

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Base Price

Starting at:

$2,595.00 Domestic price Cryo Recovery

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Details

Detailed Description

These BAC SA transgenic mice express a mutant human HTT (huntingtin) gene containing nucleotide substitutions that result in amino acid substitutions of alanine for serine at positions 13 and 16, as well as a loxP-flanked fragment containing 97 mixed CAA-CAG repeats. The amino acid substitutions render the NT17 domain phosphoresistant (SA). Immunoprecipitation analysis confirmed that the mutant HTT protein is not phosphorylated. The mutant protein is expressed at a level higher than levels detected in the BACHD-L transgenic strain (STOCK No. 017487). At 2 months of age, BAC SA transgenic mice exhibit motor deficits, which progress with age. At 12 months of age, the mutant mice display decreased forebrain weight, anxiety and depression-like behavior and progressive protein aggregate formation in cortex and striatum. Mice that are hemizygous for the targeted mutation are viable, fertile and normal in size. The Donating Investigator has not attempted to make the strain homozygous.

When bred to Htt deficient mice, the transgene rescues the null embryonic lethal phenotype.

Development

The 240 kb human bacterial artificial chromosome (BAC) RP11-866L6 containing the entire 170kb human HTT gene, and approximately 20 kbp of 5' and 50 kbp of 3' flanking sequences, was modified by nucleotide substitutions that result in amino acid substitutions of alanine for serine at positions 13 and 16, as well as replacing exon 1 with a loxP-flanked fragment containing 97 mixed CAA-CAG repeats. The SA transgenic construct was microinjected into the pronucleus of FVB/N fertilized eggs. Founder line 1 was subsequently established and maintained on the FVB/NJ background. Upon arrival at The Jackson Laboratory, the mice were crossed to FVB/NJ (Stock No. 001800) at least once to establish the colony.

Expression Data

Expressed Gene	HTT, huntingtin, human
Site of Expression

Control Suggestions

Noncarrier
001800 FVB/NJ

Additional Information

Considerations for Choosing Controls

Selected References

Gu X; Greiner ER; Mishra R; Kodali R; Osmand A; Finkbeiner S; Steffan JS; Thompson LM; Wetzel R; Yang XW. 2009. Serines 13 and 16 are critical determinants of full-length human mutant huntingtin induced disease pathogenesis in HD mice. Neuron 64(6):828-40. PubMed: 20064390 MGI: J:157723

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Genetics

Tg(HTT*)1Xwy

Allele Symbol: Tg(HTT*)1Xwy

Allele Name	transgene insertion 1, X William Yang
Allele Type	Transgenic (Humanized sequence, Inserted expressed sequence)
Allele Synonym(s)	SA
Gene Symbol and Name	Tg(HTT*)1Xwy, transgene insertion 1, X William Yang
Gene Synonym(s)	SA
Promoter	HTT, huntingtin, human
Expressed Gene	HTT, huntingtin, human
Strain of Origin	FVB
Chromosome	UN
Molecular Note	The 240 kb human bacterial artificial chromosome BAC(RP11-866L6), containing the entire 170 kb human Huntingtin (htt) genomic locus and approximately 20 kbp of 5' and 50 kbp of 3' flanking sequences, was modified by replacing the human htt exon 1 with a loxP-flanked human mutant htt exon 1 sequence (containing 97 mixed CAA-CAG repeats encoding a continuous polyglutamine (polyQ) stretch). Nucleotide substitution resulted in amino acid substitution of alanine for serine at positions 13 and 16. One line was created (no line number provided).
Mutations Made By	X. William Yang, University of California Los Angeles

Disease/Phenotype

Disease Terms

Research Areas By Genotype

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

Neurobiology Research
- Ataxia (Movement) Defects
- Behavioral and Learning Defects
  - high anxiety
- Huntington's disease
- Neurodegeneration

Genotype: HTT related

Developmental Biology Research
- Neurodevelopmental Defects
Neurobiology Research
- Ataxia (Movement) Defects
- Behavioral and Learning Defects
- Cortical Defects
- Huntington's disease
- Neurodegeneration
- Neurodevelopmental Defects
- Neurotransmitter Receptor and Synaptic Vesicle Defects
- Tremor Defects

Mammalian Phenotype Terms by Genotype

Genotype: Tg(HTT)1Xwy/0
FVB-Tg(HTT)1Xwy

nervous system phenotype

abnormal cerebral cortex morphology
- mice exhibit a 17% reduction in cortical volume compared with wild-type mice

abnormal forebrain morphology
- mice exhibit decreased forebrain weight compared with wild-type mice

abnormal neuron morphology
- at 12 months, mice exhibit protein aggregates in the cortex and striatum unlike in wild-type mice
- at 2 months, mice exhibit protein aggregates that worsen with age unlike in wild-type mice

decreased striatum area
- mice exhibit a 17% reduction in striatal volume compared with wild-type mice

neuron degeneration
- at 12 months, mice exhibit selective neuropathy unlike wild-type mice

behavior/neurological phenotype

abnormal depression-related behavior
- at 12 months, mice exhibit depressive-like behavior unlike wild-type mice

impaired coordination
- starting at 2 months and worsening with age

increased anxiety-related response
- mice spend less time in the light portion of a light/dark box than wild-type mice

References

Gu X; Greiner ER; Mishra R; Kodali R; Osmand A; Finkbeiner S; Steffan JS; Thompson LM; Wetzel R; Yang XW. 2009. Serines 13 and 16 are critical determinants of full-length human mutant huntingtin induced disease pathogenesis in HD mice. Neuron 64(6):828-40. PubMed: 20064390 MGI: J:157723

Pricing & Availability

Cryo Recovery

Domestic
International

Live Mouse

Age

Genotype

Price

weeks

Cryorecovery - Pricing

Service	Genotype	Price
	Hemizygous or Non carrier for Tg(HTT*)1Xwy

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

$2,595.00 per straw or vial

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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.

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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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Licensing Information

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Also Known As: BAC SA

Donating Investigator

Genetic overview

Research Applications

Base Price

Detailed Description

Development

Expression Data

Control Suggestions

Additional Information

Selected References

Tg(HTT*)1Xwy

Allele Symbol: Tg(HTT*)1Xwy

Disease Terms

Research Areas By Genotype

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

Genotype: HTT related

Mammalian Phenotype Terms by Genotype

Genotype: Tg(HTT*)1Xwy/0FVB-Tg(HTT*)1Xwy

nervous system phenotype

behavior/neurological phenotype

References

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

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

Genotype: Tg(HTT)1Xwy/0
FVB-Tg(HTT)1Xwy