All Phenotypes Dmd<mdx-3Cv> MGI Mouse

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Phenotypes associated with this allele

Allele Symbol
Allele Name
Allele ID

Dmd^mdx-3Cv
X linked muscular dystrophy 3, Verne Chapman
MGI:1856330

Summary

7 genotypes

Jump to	Allelic Composition	Genetic Background	Genotype ID
hm1	Dmd^mdx-3Cv/Dmd^mdx-3Cv	B6.Cg-Dmd^mdx-3Cv/J	MGI:3798631
hm2	Dmd^mdx-3Cv/Dmd^mdx-3Cv	B6Ros.Cg-Dmd^mdx-3Cv	MGI:3798616
cx3	Dmd^mdx-3Cv/Dmd^mdx-3Cv Utrn^tm1Ked/Utrn^tm1Ked	involves: 129 * C3H/HeHa * C57BL * M. m. castaneus * M. m. musculus	MGI:3798662
cx4	Dmd^mdx-3Cv/Y Utrn^tm1Ked/Utrn^tm1Ked	involves: 129 * C3H/HeHa * C57BL * M. m. castaneus * M. m. musculus	MGI:3798665
ot5	Dmd^mdx-3Cv/Y	B6.Cg-Dmd^mdx-3Cv/J	MGI:3798632
ot6	Dmd^mdx-3Cv/Y	B6Ros.Cg-Dmd^mdx-3Cv	MGI:3798617
ot7	Dmd^mdx-3Cv/Y	involves: C3H/HeHa * C57BL/6Ros * C57BL/10Sn * M. m. castaneus * M. m. musculus	MGI:3798606

Allelic Composition	Dmd^mdx-3Cv/Dmd^mdx-3Cv
Genetic Background	B6.Cg-Dmd^mdx-3Cv/J

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Dmd^mdx-3Cv mutation (1 available); any Dmd mutation (154 available)

♀	phenotype observed in females
♂	phenotype observed in males
N	normal phenotype

nervous system

abnormal Muller cell morphology ( J:125566 )

• mice exhibit an alteration in Muller cell current profile such that the current is weakest in the end feet with a gradual increase moving up the proximal process unlike in wild-type cells

vision/eye

abnormal Muller cell morphology ( J:125566 )

• mice exhibit an alteration in Muller cell current profile such that the current is weakest in the end feet with a gradual increase moving up the proximal process unlike in wild-type cells

Allelic Composition	Dmd^mdx-3Cv/Dmd^mdx-3Cv
Genetic Background	B6Ros.Cg-Dmd^mdx-3Cv

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Dmd^mdx-3Cv mutation (1 available); any Dmd mutation (154 available)

♀	phenotype observed in females
♂	phenotype observed in males
N	normal phenotype

mortality/aging

preweaning lethality, incomplete penetrance ( J:12150 )

• fewer than expected mice survive until weaning compared to Dmd^mdx mice

reproductive system

reduced fertility ( J:12150 )

• fewer than exected mice are produced compared to Dmd^mdx mice

decreased litter size ( J:12150 )

muscle

increased variability of skeletal muscle fiber size ( J:12150 )

• in mutant mice, myofibers contain fibers of different diameters with centrally located nuclei

centrally nucleated skeletal muscle fibers ( J:23502 )

• 70% of myofibers contain central nuclei unlike in wild-type mice

abnormal diaphragm morphology ( J:23502 )

• mutant mice exhibit fibrosis, fatty infiltration and necrosis in the diaphragm with increased severity upon aging

skeletal muscle fibrosis ( J:23502 )

• mice exhibit fibrosis, fatty infiltration and necrosis in the diaphragm unlike in wild-type mice that increases with age

Allelic Composition	Dmd^mdx-3Cv/Dmd^mdx-3Cv Utrn^tm1Ked/Utrn^tm1Ked
Genetic Background	involves: 129 * C3H/HeHa * C57BL * M. m. castaneus * M. m. musculus

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Dmd^mdx-3Cv mutation (1 available); any Dmd mutation (154 available)
Utrn^tm1Ked mutation (7 available); any Utrn mutation (304 available)

♀	phenotype observed in females
♂	phenotype observed in males
N	normal phenotype

muscle

abnormal skeletal muscle morphology ( J:116348 )

• authors state that mice develop the same skeletal muscle abnormalities as observed in Dmd^mdx Utrn^tm1Ked mice

nervous system

nervous system phenotype ( J:116348 )

N	• unlike in Dmd^mdx Utrn^tm1Ked mice, brain morphology is normal

respiratory system

respiratory system phenotype ( J:116348 )

N	• unlike in Dmd^mdx Utrn^tm1Ked mice, lung morphology is normal

liver/biliary system

liver/biliary system phenotype ( J:116348 )

N	• liver morphology is normal

renal/urinary system

renal/urinary system phenotype ( J:116348 )

N	• kidney morphology is normal

reproductive system

reproductive system phenotype ( J:116348 )

N	• ovary morphology is normal

Allelic Composition	Dmd^mdx-3Cv/Y Utrn^tm1Ked/Utrn^tm1Ked
Genetic Background	involves: 129 * C3H/HeHa * C57BL * M. m. castaneus * M. m. musculus

Find Mice

♀	phenotype observed in females
♂	phenotype observed in males
N	normal phenotype

muscle

abnormal skeletal muscle morphology ( J:116348 )

• authors state that mice develop the same skeletal muscle abnormalities as observed in Dmd^mdx Utrn^tm1Ked mice

nervous system

nervous system phenotype ( J:116348 )

N	• unlike in Dmd^mdx Utrn^tm1Ked mice, brain morphology is normal

respiratory system

respiratory system phenotype ( J:116348 )

N	• unlike in Dmd^mdx Utrn^tm1Ked mice, lung morphology is normal

liver/biliary system

liver/biliary system phenotype ( J:116348 )

N	• liver morphology is normal

renal/urinary system

renal/urinary system phenotype ( J:116348 )

N	• kidney morphology is normal

reproductive system

reproductive system phenotype ( J:116348 )

N	• testis morphology is normal

Allelic Composition	Dmd^mdx-3Cv/Y
Genetic Background	B6.Cg-Dmd^mdx-3Cv/J

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Dmd^mdx-3Cv mutation (1 available); any Dmd mutation (154 available)

♀	phenotype observed in females
♂	phenotype observed in males
N	normal phenotype

muscle

abnormal skeletal muscle morphology ( J:134272 )

• skeletal muscles exhibit variation in fiber size and centrally located nuclei unlike in wild-type mice

• mice exhibit macrophage invasion into muscles unlike in wild-type mice

increased variability of skeletal muscle fiber size ( J:134272 )

centrally nucleated skeletal muscle fibers ( J:134272 )

abnormal muscle contractility ( J:134272 )

• highest specific tetanic force is lower than in wild-type mice

• at high frequencies mice titanic force is greater than in Dmd^mdx-4Cv mice

• following eccentric contraction damage, force is reduced to unlike in wild-type mice that experience only a moderate drop in force but not as severely as in Dmd^mdx-4Cv mice

• age exacerbates force losses

vision/eye

vision/eye phenotype ( J:53822 )

N	• retina morphology is normal

abnormal Muller cell morphology ( J:125566 )

• mice exhibit an alteration in Muller cell current profile such that the current is weakest in the end feet with a gradual increase moving up the proximal process unlike in wild-type cells

abnormal eye electrophysiology ( J:53822 )

• mice exhibit a severely attenuated b-wave amplitude compared to wild-type mice

• b-wave and OP3 wave implicit times are delayed compared to in wild-type mice

• derived positive (P2) responses are delayed compared to in wild-type mice

behavior/neurological

abnormal grip strength ( J:134272 )

• mice exhibit stronger grip strength than Dmd^mdx-4Cv mice

nervous system

abnormal Muller cell morphology ( J:125566 )

• mice exhibit an alteration in Muller cell current profile such that the current is weakest in the end feet with a gradual increase moving up the proximal process unlike in wild-type cells

Allelic Composition	Dmd^mdx-3Cv/Y
Genetic Background	B6Ros.Cg-Dmd^mdx-3Cv

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Dmd^mdx-3Cv mutation (1 available); any Dmd mutation (154 available)

♀	phenotype observed in females
♂	phenotype observed in males
N	normal phenotype

mortality/aging

preweaning lethality, incomplete penetrance ( J:12150 )

• fewer than expected mice survive until weaning compared to Dmd^mdx mice

reproductive system

reduced fertility ( J:12150 )

• fewer than exected mice are produced compared to Dmd^mdx mice

decreased litter size ( J:12150 )

muscle

increased variability of skeletal muscle fiber size ( J:12150 )

• in mutant mice, myofibers contain fibers of different diameters with centrally located nuclei

centrally nucleated skeletal muscle fibers ( J:23502 )

• 70% of myofibers contain central nuclei unlike in wild-type mice

abnormal diaphragm morphology ( J:23502 )

• mutant mice exhibit fibrosis, fatty infiltration and necrosis in the diaphragm with increased severity upon aging

skeletal muscle fibrosis ( J:23502 )

• mice exhibit fibrosis, fatty infiltration and necrosis in the diaphragm unlike in wild-type mice that increases with age

Allelic Composition	Dmd^mdx-3Cv/Y
Genetic Background	involves: C3H/HeHa * C57BL/6Ros * C57BL/10Sn * M. m. castaneus * M. m. musculus

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Dmd^mdx-3Cv mutation (1 available); any Dmd mutation (154 available)

♀	phenotype observed in females
♂	phenotype observed in males
N	normal phenotype

muscle

skeletal muscle fiber degeneration ( J:9638 )

• mice exhibit skeletal muscle fiber degeneration

impaired muscle relaxation ( J:9638 )

• electromyograms reveal peudomyotonia unlike in wild-type mice

cardiovascular system

cardiac fibrosis ( J:9638 )

• unlike wild-type, some cardiac fibrosis is observed

vision/eye

abnormal eye electrophysiology ( J:23375 )

• although the a-wave amplitude is normal, the b-wave is attenuated by more than 50%

Mouse Models of Human Disease	DO ID	OMIM ID(s)	Ref(s)
Becker muscular dystrophy	DOID:9883	OMIM:300376	J:23375
Duchenne muscular dystrophy	DOID:11723	OMIM:310200	J:23375

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