Overview

Also Known As:GR-LACS/lipidosin null

GR-LACS^-/- mice have a neo cassette replacing exons 1-2 and part of exon 2 of the Acsbg1 gene, abolishing gene expression in the testis, ovary, adrenal gland, and brain. These mice may be useful for studying the mechanisms of gonadotropin-induced desensitization of testosterone production.

Donating Investigator

Maria Dufau, NIH

Genetic overview

Genetic Background	Generation

Acsbg1^tm1Mld

Allele Type	Gene Symbol	Gene Name
Targeted (Null/Knockout)	Acsbg1	acyl-CoA synthetase bubblegum family member 1

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Research Applications

Metabolism Research
Reproductive Biology Research
Cell Biology Research

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Base Price

Starting at:

$2,854.50 Domestic price Cryo Recovery

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Details

Detailed Description

In this strain, a neo cassette replaces exons 1-2 of the acyl-CoA synthetase bubblegum family member 1 (Acsbg1) gene, abolishing gene expression. ACSBG1, also known as gonadotropin-regulated long chain acyl-CoA synthetase (GR-LACS) or lipidosin, is an enzyme that is transcriptionally regulated by gonadotropin in rat testis. GR-LACS is involved in protein and lipid transport, enzyme activation, protein acylation, cell signaling and transcription, and plays a role in brain very long-chain fatty acids (VLCFA) metabolism and myelinogenesis. GR-LACS has also been associated with X-linked adrenoleukodystrophy (X-ALD), an adreno/neurodegenerative disorder with accumulation of VLCFA in tissues and plasma. Homozygous GR-LACS^-/- mice are viable and fertile. Mice have increased levels of long chain fatty acid in testis, ovary, brain, and decreased adrenal gland palmitic, oleic, and linoleic acid levels. Males exhibit nuclear inclusions in Leydig cells and increased gonadotropin induced changes in testosterone production. These mice have no apparent phenotypic abnormalities associated with X-ALD.

Development

A targeting vector was designed in the laboratory of Dr. Maria L Dufau (Molecular Endocrinology NICHD, NIH) to replace exons 1-2 of the acyl-CoA synthetase bubblegum family member 1 (Acsbg1) gene with a neomycin resistance (neo) cassette. The construct was electroporated into (129X1/SvJ x 129S1/Sv)F1-Kitl⁺ -derived R1 embryonic stem (ES) cells. Correctly targeted ES cells were injected into blastocysts and the resulting chimeric males were bred to C57BL/6N to generate a colony of GR-LACS^-/- mice. These mice were bred to C57BL/6N mice for at least 15 generations. Upon arrival at The Jackson Laboratory, mice were bred to C57BL/6NJ (Stock No. 005304) for at least one generation to establish the colony.

Control Suggestions

Wild-type from the colony
005304 C57BL/6NJ

Additional Information

Considerations for Choosing Controls

Selected References

Sheng Y; Tsai-Morris CH; Li J; Dufau ML. 2009. Lessons from the gonadotropin-regulated long chain acyl-CoA synthetase (GR-LACS) null mouse model: a role in steroidogenesis, but not result in X-ALD phenotype. J Steroid Biochem Mol Biol 114(1-2):44-56PubMed: 19167491 MGI: J:150391

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Genetics

Acsbg1^tm1Mld

Allele Symbol: Acsbg1^tm1Mld

Allele Name	targeted mutation 1, Maria L Dufau
Allele Type	Targeted (Null/Knockout)
Allele Synonym(s)
Gene Symbol and Name	Acsbg1, acyl-CoA synthetase bubblegum family member 1
Gene Synonym(s)
Strain of Origin	(129X1/SvJ x 129S1/Sv)F1-Kitl⁺
Chromosome	9
Molecular Note	A genomic fragment including exons 1 and 2 was replaced with a neo cassette via homologous recombination. Northern blot analysis confirmed the absence of RNA expression in testis from homozygous mice. Western blot analysis confirmed the absence of protein expression in the testis, ovary, adrenal gland, and brain from homozygous mice.

Disease/Phenotype

Disease Terms

No similarity to the expected human disease phenotype was found. One or more human genes are associated with this human disease. The mouse genotype may involve mutations to orthologs of one or more of these genes, but the phenotype did not resemble the disease.

adrenoleukodystrophy

Research Areas By Phenotype

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

Metabolism Research
- Lipid Metabolism
Reproductive Biology Research
- Developmental Defects Affecting Gonads
Cell Biology Research
- Signal Transduction
- Post-translational Processing

Mammalian Phenotype Terms by Genotype

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

Genotype: Acsbg1^tm1Mld/Acsbg1^tm1Mld
involves: 129S1/Sv * 129X1/SvJ * C57BL/6

reproductive system phenotype

abnormal Leydig cell morphology
- inclusions are surrounded by a single membrane and contain degenerated cytoplasmic components
- at 9 months of age, significant nuclear inclusions are seen in Leydig cells while such inclusions aren't seen in wild-type mice until 15 months of age

abnormal testis physiology
- enhanced human chorionic gonadotropin induced testosterone production in vivo and impaired gonadotropin induced desensitization in vitro

endocrine/exocrine gland phenotype

abnormal Leydig cell morphology
- inclusions are surrounded by a single membrane and contain degenerated cytoplasmic components
- at 9 months of age, significant nuclear inclusions are seen in Leydig cells while such inclusions aren't seen in wild-type mice until 15 months of age

abnormal testis physiology
- enhanced human chorionic gonadotropin induced testosterone production in vivo and impaired gonadotropin induced desensitization in vitro

homeostasis/metabolism phenotype

increased fatty acids level
- some very long chain fatty acids are increased in the brain and testis
- minor but significant increase in some long chain fatty acids in the testis, ovary and brain

increased circulating testosterone level
- Normal - however, basal levels are similar to wild-type controls
- levels are increased compared to similarly treated controls after a single dose of human chorionic gonadotropin

decreased fatty acids level
- decrease in palmitic, oleic and linoleic acid in the adrenal gland

mammalian phenotype

reproductive system phenotype
- Normal - both males and females are fertile

References

Sheng Y; Tsai-Morris CH; Li J; Dufau ML. 2009. Lessons from the gonadotropin-regulated long chain acyl-CoA synthetase (GR-LACS) null mouse model: a role in steroidogenesis, but not result in X-ALD phenotype. J Steroid Biochem Mol Biol 114(1-2):44-56PubMed: 19167491 MGI: J:150391

Additional - Acsbg1^tm1Mld related

Technical Support

CONTACT TECHNICAL SUPPORT

Genotyping Protocols
- Standard PCR:Acsbg1
- Genotyping resources and troubleshooting
Breeding Considerations

When maintaining a live colony, homozygous mice may be bred together.
- Additional Breeding and Husbandry Support
Mating System
- Heterozygote x Heterozygote
Citation
When using the GR-LACS/lipidosin null mouse strain in a publication, please cite the originating article(s) and include JAX stock #024754 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 wildtype for Acsbg1<tm1Mld>

Related Products and Services

Frozen Mouse Embryo	B6N.129-Acsbg1<tm1Mld>/J Frozen Embryo	$2595.00
Frozen Mouse Embryo	B6N.129-Acsbg1<tm1Mld>/J Frozen Embryo	$2595.00
Frozen Mouse Embryo	B6N.129-Acsbg1<tm1Mld>/J Frozen Embryo	$3373.50
Frozen Mouse Embryo	B6N.129-Acsbg1<tm1Mld>/J Frozen Embryo	$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.

Terms Of Use

General Terms and Conditions

Questions about Terms of Use

Licensing Information

Phone: 207-288-6470

Email: TechTran@jax.org

Also Known As:GR-LACS/lipidosin null

Donating Investigator

Genetic overview

Research Applications

Base Price

Detailed Description

Development

Control Suggestions

Additional Information

Selected References

Acsbg1tm1Mld

Allele Symbol: Acsbg1tm1Mld

Disease Terms

No similarity to the expected human disease phenotype was found. One or more human genes are associated with this human disease. The mouse genotype may involve mutations to orthologs of one or more of these genes, but the phenotype did not resemble the disease.

Research Areas By Phenotype

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

Mammalian Phenotype Terms by Genotype

Genotype: Acsbg1tm1Mld/Acsbg1tm1Mldinvolves: 129S1/Sv * 129X1/SvJ * C57BL/6

reproductive system phenotype

endocrine/exocrine gland phenotype

homeostasis/metabolism phenotype

mammalian phenotype

References

Additional - Acsbg1tm1Mld related

Genotyping Protocols

Breeding Considerations

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

Acsbg1^tm1Mld

Allele Symbol: Acsbg1^tm1Mld

Genotype: Acsbg1^tm1Mld/Acsbg1^tm1Mld
involves: 129S1/Sv * 129X1/SvJ * C57BL/6

Additional - Acsbg1^tm1Mld related