Dmd;mdx;Utrn

There is no network for this step.

Fullscreen mode

Hide graph

Legend

Genetic mutants with Dmd, mdx, Utrn alterations

Names of genes are ordered alphabetically. For the order of interventions, please see the specific paper.

Lifespan (days)
427.0
Lifespan comparisons
Triple mutant DMD(OE);mdx(KO);utrn(KO) has a lifespan of 427.0 days, while double mutant mdx(KO);utrn(KO) has a lifespan of 274.0 days and wild type has a lifespan of 427.0 days.
Citation
View abstract
Gaedigk R et al., 2006, Improvement in survival and muscle function in an mdx/utrn(-/-) double mutant mouse using a human retinal dystrophin transgene. Neuromuscul Disord. 16(3):192-203 16487708 Click here to select all mutants from this PubMed ID in the graph
Abstract
Duchenne muscular dystrophy is a progressive muscle disease characterized by increasing muscle weakness and death by the third decade. mdx mice exhibit the underlying muscle disease but appear physically normal with ordinary lifespans, possibly due to compensatory expression of utrophin. In contrast, double mutant mice (mdx/utrn(-/-)), deficient for both dystrophin and utrophin die by approximately 3 months and suffer from severe muscle weakness, growth retardation, and severe spinal curvature. The capacity of human retinal dystrophin (Dp260) to compensate for the missing 427 kDa muscle dystrophin was tested in mdx/utrn(-/-) mice. Functional outcomes were assessed by histology, EMG, MRI, mobility, weight and longevity. MCK-driven transgenic expression of Dp260 in mdx/utrn(-/-) mice converts their disease course from a severe, lethal muscular dystrophy to a viable, mild myopathic phenotype. This finding is relevant to the design of exon-skipping therapeutic strategies since Dp260 lacks dystrophin exons 1-29.

Lifespan (days)
427.0
Lifespan comparisons
Triple mutant DMD(OE);mdx(KO);utrn(KO) has a lifespan of 427.0 days, while double mutant mdx(KO);utrn(KO) has a lifespan of 274.0 days and wild type has a lifespan of 427.0 days.
Citation
View abstract
Gaedigk R et al., 2006, Improvement in survival and muscle function in an mdx/utrn(-/-) double mutant mouse using a human retinal dystrophin transgene. Neuromuscul Disord. 16(3):192-203 16487708 Click here to select all mutants from this PubMed ID in the graph
Abstract
Duchenne muscular dystrophy is a progressive muscle disease characterized by increasing muscle weakness and death by the third decade. mdx mice exhibit the underlying muscle disease but appear physically normal with ordinary lifespans, possibly due to compensatory expression of utrophin. In contrast, double mutant mice (mdx/utrn(-/-)), deficient for both dystrophin and utrophin die by approximately 3 months and suffer from severe muscle weakness, growth retardation, and severe spinal curvature. The capacity of human retinal dystrophin (Dp260) to compensate for the missing 427 kDa muscle dystrophin was tested in mdx/utrn(-/-) mice. Functional outcomes were assessed by histology, EMG, MRI, mobility, weight and longevity. MCK-driven transgenic expression of Dp260 in mdx/utrn(-/-) mice converts their disease course from a severe, lethal muscular dystrophy to a viable, mild myopathic phenotype. This finding is relevant to the design of exon-skipping therapeutic strategies since Dp260 lacks dystrophin exons 1-29.

Search genes: Dmd mdx Utrn Dmd;mdx;Utrn

Entrez ID
Symbol
GenAge
MGI ID

13405
Dmd
View on GenAge (Dmd)
MGI:94909

dystrophin, muscular dystrophy

Symbol
GenAge

mdx
View on GenAge (mdx)

No gene information for mdx

Entrez ID
Symbol
GenAge
MGI ID

22288
Utrn
View on GenAge (Utrn)
MGI:104631

utrophin

Orthologs of Dmd;mdx;Utrn in SynergyAge

Show in SynergyAge
Species	Gene

Not in SynergyAge
Species	Gene

Orthologs of Dmd in SynergyAge

Show in SynergyAge
Species	Gene

Orthologs of mdx in SynergyAge

Show in SynergyAge
Species	Gene

Orthologs of Utrn in SynergyAge

Show in SynergyAge
Species	Gene

About

SynergyAge database hosts high-quality, manually curated information about the synergistic and antagonistic lifespan effects of genetic interventions in model organisms, also allowing users to explore the longevity relationships between genes in a visual way.