The full-length, human dystrophin gene (with regions of Repeat 4 through Repeat 23 and the C-terminus deleted) under the transcriptional control of the human actin alpha 1, skeletal muscle promoter, was inserted randomly. Endogenous Dmd carries a premature stop codon mutation.
HGVS nomenclature (Dmdmdx allele):
Homozygous: The homozygous model of this strain exhibits restored skeletal muscle function. It also shows improved histopathology of the muscle compared to the Dmdmdx mouse.
Hemizygous: Not studied.
Li, D., Yue, Y., Lai, Y., Hakim, C. H. and Duan, D. (2011), Nitrosative stress elicited by nNOSu delocalization inhibits muscle force in dystrophin-null mice. J Pathol. 2011 Jan; 223(1): 88-98. (Medline PMC: 3109084)
Bulfield G, Siller WG, Wight PA, Moore KJ. X chromosome-linked musculardystrophy (mdx) in the mouse. Proc Natl Acad Sci U S A. 1984 Feb;81(4):1189-92.(Medline PMID: 6583703)
|Viability and Fertility:||Female||Male||Comments|
|Homozygotes are viable:||Yes||Yes|
|Homozygotes are fertile:||Yes||Yes|
|Heterozygotes are fertile:||Yes||Yes|
|Age Reproductive Decline:||Undetermined||Undetermined|
The donor or their institution limits the distribution to non-profit institutions only.
|MMRRC Item #||Description||Distribution Fee / Unit (US $)
*Shipping & Handling not included*
|041193-JAX-SPERM||Cryo-preserved spermatozoa||$437.00 / Non-Profit||Aliquot||Approximate quantity3|
|041193-JAX-RESUS||Litter recovered from cryo-archive||$2,022.00 / Non-Profit||Litter||Recovered litter4; additional fees for any special requests.|
|Cryopreserved material may be available upon request, please inquire to firstname.lastname@example.org for more information.|