Loading Mouse GIF
Loading...

Strain Name:
B6.Cg-Tg(CAG-EGFP/Map1lc3b)53Nmz Gaatm1Rabn/RabnMmjax
Stock Number:
071275-JAX
Citation ID:
RRID:MMRRC_071275-JAX
Other Names:
GFP:LC3-GAA-/-

Strain Information

Gaatm1.1Rabn
Name: glucosidase, alpha, acid; targeted mutation 1.1, Nina Raben
Synonyms: delta6
Type: Allele
Species: Mus musculus (mouse)
Chromosome: 11
Alteration at locus: Knockout
Gaa
Name: glucosidase, alpha, acid
Synonyms: E430018M07Rik
Type: Gene
Species: Mouse
Chromosome: 11
Alteration at locus: Knockout
NCBI: 14387
HGNC: HGNC:4065
Homologene: 37268
Tg(CAG-EGFP/Map1lc3b)53Nmz
Name: transgene insertion 53, Noboru Mizushima
Synonyms: GFP-LC3, Tg(CAG-EGFP/Map1lc3b)53Nmz, CAG-GFP-LC3, LC3-GFP, Tg(CAG-EGFP/M1lc3b)53Nmz, Tg(ACTB-EGFP/Map1lc3b)53Nmz, GFP-LC3B, GFP-LC3#53
Type: Allele
Species: Multi-species
Chromosome: 2
Alteration at locus: Transgenic
CAG
Name: CMV early enhancer/chicken beta actin promoter/rabbit beta globin splice acceptor
Type: DNA Segment
Species: Multi-species
Chromosome:
Alteration at locus: Transgenic
Map1lc3b
Name: microtubule-associated protein 1 light chain 3 beta
Synonyms: 1010001C15Rik, Map1lc3, LC3b, Atg8
Type: Gene
Species: Mouse
Chromosome: 8
Alteration at locus: Transgenic
NCBI: 67443
Homologene: 69359
Genetic Alterations
GFP:LC3-GAA-/- mice carry a null allele (neo inserted into exon 6) of Gaa (glucosidase, alpha, acid) and express an EGFP reporter fused to Map1lc3b (microtubule-associated protein 1 light chain 3 beta or LC3b). The reporter labels autophagosomes in a mouse model of glycogen storage disease type II (Pompe disease). This strain maybe useful in studies of lysosomal and autophagic abnormalities and lysosomal storage disorders.
ES Cell Line
129Sv
Phenotype
Muscle weakness and wasting at 7-9 months of age

Conditional phenotype: no

MeSH Terms
  • Adenoviridae/genetics
  • Animals
  • Autophagy
  • Basic Helix-Loop-Helix Leucine Zipper Transcription Factors/chemistry
  • Basic Helix-Loop-Helix Leucine Zipper Transcription Factors/genetics
  • Basic Helix-Loop-Helix Leucine Zipper Transcription Factors/metabolism
  • Cells, Cultured
  • Disease Models, Animal
  • Exocytosis
  • Genetic Vectors/genetics
  • Genetic Vectors/metabolism
  • Glycogen/metabolism
  • Glycogen Storage Disease Type II/enzymology
  • Glycogen Storage Disease Type II/pathology
  • Lysosomes/metabolism
  • Mice
  • Mice, Knockout
  • Muscle, Skeletal/cytology
  • Muscle, Skeletal/metabolism
  • Muscle, Skeletal/ultrastructure
  • alpha-Glucosidases/deficiency
  • alpha-Glucosidases/genetics
  • alpha-Glucosidases/metabolism
Strain Development
The GFP:LC3-GAA-/- strain was created in the laboratory of Dr. Nina Raben (NIAMS, NIH) by crossing mice the carrying Gaatm1Rabn allele and mice carrying Tg(CAG-EGFP/Map1lc3b)53Nmz allele. Upon arrival, sperm was cryopreserved. To establish our live colony, an aliquot of frozen sperm was used to fertilize C57BL/6NJ oocytes.

The Gaa targeting vector contains a loxP site in intron 5, a new termination codon, a new EcoRV site, a neomycin cassette and a second loxP in intron 6 of the Gaa (glucosidase, alpha, acid) gene. The construct was electroporated into 129X1/SvJ-derived RW-4 embryonic stem (ES) cells. Correctly targeted ES cells were injected into blastocysts and the resulting chimeric males were bred with C57BL/6 females.The strain was backcrossed to C57BL/6 for at least 5 generations.

The GFP:LC3 transgenic construct contains the widespread CAG promoter (cytomegalovirus immediate-early enhancer followed by a 1.3-kb DNA segment including the promoter, first exon and first intron of the chicken beta-actin gene, with the 3' splice junction sequence replaced by that of the rabbit hemoglobin beta gene) directing expression an EGFP reporter fused to the N-terminus of rat Map1lc3b (microtubule-associated protein 1 light chain 3 beta) followed by SV40 polyA sequence. The linearized construct was microinjected into the pronuclei of fertilized C57BL/6NCrj x BXDF1 fertilized eggs. Founder 53 was crossed to C57BL/6NCrj for at least 5 generations. Copy number was not determined. The transgene insertion site is located in the distal region of chromosome 2, 106 bp upstream of the Gm14314 locus.
Suggested Control Mice
GAA knockout and GFP:LC3-WT
MMRRC Genetic QC Summary
The MMRRC Centers have developed a genetic QC pipeline using MiniMUGA array genotyping to provide additional information on strain backgrounds for MMRRC congenic and inbred strains. For more information on when data may be available, or to request genotyping for a strain of interest, please contact csmmrrc@jax.org. Older strains may not have this information.
  • Cell Biology
  • Metabolism
  • Models for Human Disease
Donor
Nina Raben-Belenky, M.D., NIH.
Primary Reference

Spampanato C, Feeney E, Li L, Cardone M, Lim JA, Annunziata F, Zare H, Polishchuk R, Puertollano R, Parenti G, Ballabio A, Raben N. Transcription factor EB (TFEB) is a new therapeutic target for Pompe disease. EMBO Mol Med. 2013 May;5(5):691-706. doi: 10.1002/emmm.201202176. Epub 2013 Apr 18. (Medline PMID: 23606558)

Colony and Husbandry Information

For more information about this colony's health status contact csmmrrc@jax.org
Coat Color
Agouti
Eye
Black
MMRRC Breeding System
Sib-mating
Generation
N/A
Overall Breeding Performance
Good
NOTE: "Hemizygote" as used here refers to males carrying a mutation on the X Chromosome or mice of either sex carrying an inserted transgene with no homologous allele on the other chromosome.
Viability and Fertility: Female Male Comments
Homozygotes are viable: Yes Yes
Homozygotes are fertile: Yes Yes
Hetero/Hemizygotes are fertile: Yes Yes
Age Reproductive Decline: 7 to 9 months 7 to 9 months
Average litter size
4 to 6
Recommended wean age
3 Weeks
Average Pups Weaned
4 to 6

Order Request Information

Limited quantities of breeder mice (up to 2 males and 2 females or 4 mice) per investigator per month are available from a live colony, usually available to ship in under 12 weeks. Larger quantities may be available, please contact the distributing center directly at csmmrrc@jax.org for more details.

Cryopreserved material may be available upon request, please inquire to csmmrrc@jax.org for more information.

A Commercial License Agreement from the Donor is required for for-profit entities to use this strain. For more information, please contact Vlado Knezevic.

Distribution of this strain requires submission of the MMRRC Conditions of Use (COU). A link to the COU web form will be provided via email after an order has been placed; the form should be completed then or the email forwarded to your institutional official for completion.

A Commercial License Agreement from the Donor is required for for-profit entities to use this strain. For more information, please contact Vlado Knezevic

Additional charges may apply for any special requests. Shipping costs are in addition to the basic distribution/resuscitation fees. Information on shipping costs and any additional charges will be provided by the supplying MMRRC facility.

Click button to Request this one strain. (Use the MMRRC Catalog Search to request more than one strain.)
MMRRC Item # Description Distribution Fee / Unit (US $)
*Shipping & Handling not included*
Units Notes
071275-JAX-M01-F
071275-JAX- M01-M
071275-JAX- M02-F
071275-JAX- M02-M
Hemizygous for Tg(CAG-EGFP/Map1lc3b)53Nmz/Homozygous for Gaatm1Rabn
Hemizygous for Tg(CAG-EGFP/Map1lc3b)53Nmz/Homozygous for Gaatm1Rabn
Hemizygous for Tg(CAG-EGFP/Map1lc3b)53Nmz/Heterozygous for Gaatm1Rabn
Hemizygous for Tg(CAG-EGFP/Map1lc3b)53Nmz/Heterozygous for Gaatm1Rabn
$229.00 / $229.00
Non-Profit / For-Profit
Per Mouse The csmmrrc@jax.org may assess additional fees for any special requests (e.g., specific age or weight of mice, etc.).
Cryopreserved material may be available upon request, please inquire to csmmrrc@jax.org for more information.

1 The distribution fee covers the expense of rederiving mice from a live mouse; you will receive the resulting litter. The litter will contain at minimum one mutant carrier; the actual number of animals and the gender and genotype ratios will vary. (Typically, multiple breeder pairs can be established from the recovered litter.) Prior to shipment, the MMRRC will provide information about the animals recovered. If you anticipate or find that you need to request specific genotypes, genders or quantities of mice in excess of what is likely from a resuscitated litter, you may discuss available options and pricing with the supplying MMRRC facility.

2 An aliquot contains a sufficient number of embryos (in one or more vials or straws and based on the transfer success rate of the MMRRC facility) to transfer into one to three recipients. The MMRRC makes no guarantee concerning embryo transfer success experienced in the recipient investigator's laboratory. Neither gender nor genotype ratios are guaranteed.

3 An aliquot is one straw or vial with sufficient sperm to recover at least one litter of mice, as per provided protocols, when performed at the MMRRC facility. The MMRRC makes no guarantee concerning the success of these procedures when performed outside the MMRRC facilities.

4 The distribution fee covers the expense of resuscitating mice from the cryo-archive; you will receive the resulting litter. The litter will contain at minimum one mutant carrier; the actual number of animals and the gender and genotype ratios will vary. (Typically, multiple breeder pairs can be established from the recovered litter.) Prior to shipment, the MMRRC will provide information about the animals recovered. If you anticipate or find that you need to request specific genotypes, genders or quantities of mice in excess of what is likely from a resuscitated litter, you may discuss available options and pricing with the supplying MMRRC facility.

To request material from the MMRRC: Please fill out our on-line request form (accessible from the catalog search results page, or click the Request this Strain button in the fees section). If you have questions or need assistance completing this form, you may call Customer Service at (800) 910-2291 (in USA or Canada) or (530) 757-5710 (international calls). Before you call, please have with you: the MMRRC item number, quantity needed, Bill-to and Ship-to contact information.