B6.Cg-Terc^tm1Rdp Dmd^mdx-4Cv/BlauJ

Stock number: 023535
Product name: B6.mdx/mTR
Strain synonyms: mdx/mTR^KO
Research areas: Cancer Research, Cardiovascular Research, Cell Biology Research, Developmental Biology Research, Hematological Research, Internal/Organ Research, Mouse/Human Gene Homologs, Neurobiology Research, Reproductive Biology Research, Research Tools

Description

The mdx/mTR^KO mouse model combines dystrophin-deficiency with telomere dysfunction/shortening, and may be a superior Duchenne muscular dystrophy model as it better recapitulates several of the human characteristics of DMD myopathology (progressive muscle weakness and damage, skeletal muscle fibrosis, diminished muscle stem cell regenerative capacity, dilated cardiomyopathy, heart failure and shortened life-span).

Allele

Symbol: Terc^tm1Rdp
Name: targeted mutation 1, Ronald DePinho
MGI accession ID: MGI:1857930
Generation method: Targeted
Attributes: Null/Knockout
Marker symbol: Terc
Marker name: telomerase RNA component
Chromosome: 3
Strain of origin: STOCK 129/Sv and C57BL/6J and SJL
Molecular note: Replacement of the entire gene with a neomycin cassette.

Allele

Symbol: Dmd^mdx-4Cv
Name: X linked muscular dystrophy 4, Verne Chapman
MGI accession ID: MGI:1856331
Generation method: Chemically induced (ENU)
Marker symbol: Dmd
Marker name: dystrophin, muscular dystrophy
Chromosome: X
Strain of origin: C3Ha.Cg-Hprt^a Pgk1^a
Molecular note: A C to T transition in exon 53 at position 7916 creates a premature stop codon.