This strain carries both a mouse alpha-sarcoglycan (Sgca) targeted mutation and a human epsilon-sarcoglycan (SGCE) transgene. Mice display no overt phenotype with normal behavior and normal muscle force generation. After mild exercise, the compound mutants show an exaggerated fatigue response. This strain may be useful in studies of muscular dystrophy and exercise-induced fatigue.
Kevin Campbell, University of Iowa
Homozygous alpha-sarcoglycan targeted mutant mice develop a progressive pathology characteristic of limb-girdle muscular dystrophy type 2D starting at one week of age. Necrosis, regeneration, central nucleation, atrophy, hypertrophy, fiber splitting and endomysial fibrosis are observed in their muscle.
When combined with a human epsilon-sarcoglycan transgene driven by a mouse muscle creatine kinase promoter specific to skeletal and heart muscle (as in this compound mutant strain) mice display no overt phenotype with normal behavior and normal muscle force generation. After mild exercise, the compound mutants show an exaggerated fatigue response. Biochemically, the mice show normal levels of nNOS (NOS1) in muscle homogenates, but variable levels in membrane fractions. By immunofluorescence, nNOS (NOS1) is not localized to all muscle membrane fibers.
The Sgca targeted allele was made by replacing exons 2 and 3 of the gene with a neomycin resistance cassette. The construct was electroporated into (129X1/SvJ x 129S1/Sv)F1- Kitl+-derived R1 embryonic stem (ES) cells. The line was backcrossed five times to C57BL/6 by the donating laboratory.
The mouse muscle creatine kinase promoter specific for skeletal and cardiac muscle drives expression of the full-length human epsilon-sarcoglycan cDNA in the transgenic portion of this strain. This is Line 1 of the transgenic line. The transgenic vector was introduced to B6SJLF1 embryos. The mutation was backcrossed twice to C57BL/6 then crossed to the Sgca line mentioned above for 13 generations by the donating laboratory.
|Expressed Gene||SGCE, sarcoglycan epsilon, human|
|Site of Expression|
|Site of Expression|
|Allele Name||transgene insertion 1, Kevin Campbell|
|Allele Type||Transgenic (Inserted expressed sequence, Humanized sequence)|
|Allele Synonym(s)||Tg(Ckm-SGCE)1Kcam; transgene insertion 1, Kevin Campbell|
|Gene Symbol and Name||Tg(Ckm-SGCE)1Kcam, transgene insertion 1, Kevin Campbell|
|Promoter||Ckm, creatine kinase, muscle, mouse, laboratory|
|Expressed Gene||SGCE, sarcoglycan epsilon, human|
|Strain of Origin||(C57BL/6 x SJL)F1|
|Molecular Note||The mouse muscle creatine kinase promoter specific for skeletal and cardiac muscle drives expression of the full-length human epsilon sarcoglycan in this transgene construct.|
|Allele Name||targeted mutation 1, Kevin P Campbell|
|Allele Type||Targeted (Null/Knockout)|
|Allele Synonym(s)||Sgcatm1Kcam; targeted mutation 1, Kevin P Campbell|
|Gene Symbol and Name||Sgca, sarcoglycan, alpha (dystrophin-associated glycoprotein)|
|Gene Synonym(s)||adhalin; ADL; 50DAG; DAG2; DMDA2; LGMD2D; SCARMD1; Asg; LGMDR3|
|Strain of Origin||(129X1/SvJ x 129S1/Sv)F1-Kitl+|
|Molecular Note||Replacement of 902 bp including exons 2 and 3 with a neomycin cassette. No mRNA was detectable in Northern blots of total RNA derived from skeletal muscle tissue of homozygous mutants, and no protein was detected in Western blots in membrane enriched preparations of skeletal muscle.|
The donating investigator crosses homozygous Sgca targeted mutant/hemizygous SGCE transgenic males with homozygous Sgca targeted mutant females to maintain a colony.
When using the B6.Cg-Sgcatm1Kcam Tg(Ckm-SGCE)1Kcam/J mouse strain in a publication, please cite the originating article(s) and include JAX stock #009651 in your Materials and Methods section.
|Heterozygous for Sgca<tm1Kcam> , Hemizygous or Non Carrier for Tg(Ckm-SGCE)1Kcam|
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.
|Frozen Mouse Embryo||$2,595.00 per straw or vial|
"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.
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.
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.
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.
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.