All Phenotypes Dmd<mdx-5Cv> MGI Mouse

Phenotypes associated with this allele

Jump to	Allelic Composition	Genetic Background	Genotype ID
hm1	Dmd^mdx-5Cv/Dmd^mdx-5Cv	B6Ros.Cg-Dmd^mdx-5Cv	MGI:3798620
hm2	Dmd^mdx-5Cv/Dmd^mdx-5Cv	B6Ros.Cg-Dmd^mdx-5Cv/J	MGI:3798788
cx3	Dmd^mdx-5Cv/Dmd^mdx-5Cv Flt1^tm1Jrt/Flt1⁺ Utrn^tm1Jrs/Utrn^tm1Jrs	involves: 129S1/Sv * 129X1/SvJ * C3HA * C57BL/6Ros	MGI:4844311
cx4	Dmd^mdx-5Cv/Dmd^mdx-5Cv Flt1^tm1Jrt/Flt1⁺	involves: C3HA * C57BL/6Ros	MGI:4844310
ot5	Dmd^mdx-5Cv/Y	B6Ros.Cg-Dmd^mdx-5Cv	MGI:3798621

Allelic Composition	Dmd^mdx-5Cv/Dmd^mdx-5Cv
Genetic Background	B6Ros.Cg-Dmd^mdx-5Cv

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Dmd^mdx-5Cv mutation (1 available); any Dmd mutation (154 available)

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

muscle

abnormal diaphragm morphology ( J:23502 )

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

skeletal muscle fibrosis ( J:23502 )

• mice exhibit progressive fibrosis of the diaphragm with age, unlike in wild-type mice

Allelic Composition	Dmd^mdx-5Cv/Dmd^mdx-5Cv
Genetic Background	B6Ros.Cg-Dmd^mdx-5Cv/J

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Dmd^mdx-5Cv mutation (1 available); any Dmd mutation (154 available)

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

cardiovascular system

cardiovascular system phenotype ( J:124861 )

N	• despite defects in conduction, mice do not exhibit cardiac fibrosis or cellular hypertrophy in cardiac tissues • heart rate and ventricular repolarization, reflected on the mouse ECG by the ST interval, are similar to controls

decreased P wave amplitude ( J:124861 )

• decreased amplitude and prolonged duration

prolonged P wave ( J:124861 )

prolonged QRS complex duration ( J:124861 )

• prolonged

abnormal myocardial fiber physiology ( J:124861 )

• sodium current is decreased compared to in wild-type cardiomyocytes

muscle

increased variability of skeletal muscle fiber size ( J:164891 )

centrally nucleated skeletal muscle fibers ( J:164891 )

increased satellite cell number ( J:164891 )

• compared with wild-type mice

abnormal muscle contractility ( J:164891 )

• mice exhibit reduced muscle contractile function compared with wild-type mice

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
Duchenne muscular dystrophy		DOID:11723	OMIM:310200	J:124861

Allelic Composition	Dmd^mdx-5Cv/Dmd^mdx-5Cv Flt1^tm1Jrt/Flt1⁺ Utrn^tm1Jrs/Utrn^tm1Jrs
Genetic Background	involves: 129S1/Sv * 129X1/SvJ * C3HA * C57BL/6Ros

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Dmd^mdx-5Cv mutation (1 available); any Dmd mutation (154 available)
Flt1^tm1Jrt mutation (1 available); any Flt1 mutation (76 available)
Utrn^tm1Jrs mutation (2 available); any Utrn mutation (304 available)

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

mortality/aging

extended life span ( J:164891 )

• compared with Dmd^mdx-5Cv Utrn^tm1Jrs homozygotes

muscle

centrally nucleated skeletal muscle fibers ( J:164891 )

• mice exhibit fewer muscle fibers with centrally localized nuclei compared with Dmd^mdx-5Cv Utrn^tm1Jrs homozygotes

growth/size/body

increased body weight ( J:164891 )

• compared with Dmd^mdx-5Cv Utrn^tm1Jrs homozygotes

Allelic Composition	Dmd^mdx-5Cv/Dmd^mdx-5Cv Flt1^tm1Jrt/Flt1⁺
Genetic Background	involves: C3HA * C57BL/6Ros

Find Mice

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

muscle

muscle phenotype ( J:164891 )

N	• mice exhibit more stable and less degenerative muscle fibers with fewer calcium deposits and reduced fibrosis in the diaphragm compared with Dmd^mdx-5Cv homozygotes

abnormal muscle development ( J:164891 )

• mice exhibit an increase in total satellite cells and myogenic precursor cells compared with Dmd^mdx-5Cv homozygotes

abnormal skeletal muscle fiber morphology ( J:164891 )

• mice exhibit an increase in the number of slow muscle fibers compared with Dmd^mdx-5Cv homozygotes

abnormal skeletal muscle fiber size ( J:164891 )

• mice exhibit less variability in skeletal muscle fiber size compared with Dmd^mdx-5Cv homozygotes

increased satellite cell number ( J:164891 )

• mice exhibit an increase in total satellite cells and myogenic precursor cells compared with Dmd^mdx-5Cv homozygotes and wild-type mice

abnormal muscle contractility ( J:164891 )

• mice exhibit improved muscle contractile function compared with Dmd^mdx-5Cv homozygotes

cardiovascular system

increased capillary density ( J:164891 )

• capillary density in the tibialis anterior muscle and diaphragm is increased compared to in Dmd^mdx-5Cv homozygotes

abnormal blood circulation ( J:164891 )

• mice exhibit increased blood perfusion compared with Dmd^mdx-5Cv homozygotes

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

muscle

muscle phenotype ( J:177391 )

N	• limb and diaphragm muscle weights are similar to controls in mice at 1 year of age

abnormal diaphragm morphology ( J:23502 )

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

skeletal muscle fibrosis ( J:23502 , J:177391 )

• mice exhibit progressive fibrosis of the diaphragm with age, unlike in wild-type mice (J:23502)

• increased collagen deposition in the diaphragm and quadriceps muscles between 12 weeks and 1 year of age (J:177391)

impaired skeletal muscle contractility ( J:177391 )

• decrease in the tetanic force produced by the diaphragm muscle compared to wild-type controls and mice hemizygous for Dmd^mdx

• however, the decrease in the tetanic force produced by the extensor digitorum longus muscle is similar to mice hemizygous for Dmd^mdx

increased muscle fatigability ( J:177391 )

• the diaphragm muscle shows enhanced susceptibility to fatigue in a repetitive isometric contraction protocol compared to wild-type controls and mice hemizygous for Dmd^mdx

behavior/neurological

impaired coordination ( J:177391 )

• significant motor deficit is detected in a rotarod assay at 6 - 20 weeks of age

decreased grip strength ( J:177391 )

• in fore and hind paws

abnormal gait ( J:177391 )

• the hind paw does not always land on the same position as the front paw

• increased hind paw base width

short stride length ( J:177391 )

• at 8 weeks, 12 weeks and 1 year of age

fatigue ( J:177391 )

• display extreme fatigue after 15 min of downhill treadmill running

• by 8 weeks of age show maximum decreases in both ambulation and rearing events after exercise, earlier than in mice hemizygous for Dmd^mdx

• by 12 weeks of age show a decrease in distance traveled after exercise compared to wild-type controls and mice hemizygous for Dmd^mdx

• response to exercise induced fatigue is uniform across individuals unlike in mice hemizygous for Dmd^mdx

adipose tissue

decreased body fat mass ( J:177391 )

• decrease in the accumulation of abdominal fat compared to wild-type controls

growth/size/body

decreased body fat mass ( J:177391 )

• decrease in the accumulation of abdominal fat compared to wild-type controls

decreased body weight ( J:177391 )

• between 8 and 20 weeks of age compared to wild-type controls

• lower than in mice hemizygous for Dmd^mdx at most ages tested

• however, body size as determined by measures of tibia length or body length is similar to wild-type controls

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
Duchenne muscular dystrophy		DOID:11723	OMIM:310200	J:177391

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