Phenotypes associated with this allele

• Allele Symbol: Tg(ACTA1-cre/ERT2)97.16Mtz
• Allele Name: transgene insertion 97.16, Daniel Metzger
• Allele ID: MGI:3576667
• Summary: 3 genotypes

Jump to	Allelic Composition	Genetic Background	Genotype ID
cn1	Selenon^tm1.1Mred/Selenon^tm1.1Mred Tg(ACTA1-cre/ERT2)97.16Mtz/0	involves: 129S2/SvPas * C57BL/6J	MGI:5298007
cn2	Grpel1^tm1c(EUCOMM)Wtsi/Grpel1^tm1c(EUCOMM)Wtsi Tg(ACTA1-cre/ERT2)97.16Mtz/0	involves: C57BL/6N	MGI:7411110
cn3	Tg(ACTA1-cre/ERT2)97.16Mtz/0 Tg(CAG-DMPK*)1323Coop/0	involves: FVB	MGI:5426828

Genotype
MGI:5298007

cn1

• Allelic Composition: Selenon^tm1.1Mred/Selenon^tm1.1Mred; Tg(ACTA1-cre/ERT2)97.16Mtz/0
• Genetic Background: involves: 129S2/SvPas * C57BL/6J

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

normal phenotype

no abnormal phenotype detected ( J:176499 )

• mice exhibit normal post-natal development, growth, and activity

Genotype
MGI:7411110

cn2

• Allelic Composition: Grpel1^{tm1c(EUCOMM)Wtsi}/Grpel1^{tm1c(EUCOMM)Wtsi}; Tg(ACTA1-cre/ERT2)97.16Mtz/0
• Genetic Background: involves: C57BL/6N

muscle

abnormal skeletal muscle morphology ( J:330869 )

• disorganized in tamoxifen-treated mice

decreased skeletal muscle fiber diameter ( J:330869 )

• in tamoxifen-treated mice

skeletal muscle atrophy ( J:330869 )

• in tamoxifen-treated mice

skeletal muscle fibrosis ( J:330869 )

• in tamoxifen-treated mice

abnormal muscle physiology ( J:330869 )

• systemic metabolic alterations with decreased glycolysis and increased fatty acids in muscles of tamoxifen-treated mice

homeostasis/metabolism

homeostasis/metabolism phenotype ( J:330869 )

N • tamoxifen-treated mice exhibit normal oxygen consumption, respiratory exchange ratio, heat production, glucose tolerance, and insulin sensitivity

decreased circulating glucose level ( J:330869 )

• in tamoxifen-treated mice at end point

growth/size/body

weight loss ( J:330869 )

• in tamoxifen-treated mice without a change in movement, oxygen consumption, respiratory exchange ratio, or heat production

behavior/neurological

decreased grip strength ( J:330869 )

• in tamoxifen-treated mice

Genotype
MGI:5426828

cn3

• Allelic Composition: Tg(ACTA1-cre/ERT2)97.16Mtz/0; Tg(CAG-DMPK*)1323Coop/0
• Genetic Background: involves: FVB

behavior/neurological

abnormal motor capabilities/coordination/movement ( J:132154 )

• on a graded treadmill protocol, mutants treated with tamoxifen show a decrease in the time of drop off from the treadmill compared to controls

abnormal locomotor behavior ( J:132154 )

• by 4 weeks after tamoxifen administration, 70% of mutants show reduced mobility

abnormal gait ( J:132154 )

• by 4 weeks after tamoxifen administration, 70% of mutants show abnormal gait

muscle

abnormal skeletal muscle fiber morphology ( J:132154 )

• mutants treated with tamoxifen exhibit increased numbers of smaller myocytes, scattered basophilic fibers, acute myofibrillar degeneration with significant numbers of necrotic fibers and many fibers with basophilic cytoplasm and central nuclei consistent with regeneration

• four weeks after tamoxifen administration, mutants show a loss in fibers expressing solely slow twitch myosin and an increase in the number of fibers showing simultaneous expression of slow and fast twitch myosin isoforms

decreased skeletal muscle size ( J:132154 )

• by 4 weeks after tamoxifen administration, mutants show a severe reduction in muscle size

• muscle wasting progressively worsens with a 15% reduction in diameter by 4 weeks after tamoxifen administration

skeletal muscle degeneration ( J:132154 )

• mutants treated with tamoxifen exhibit severe and progressive skeletal muscle wasting and degeneration

skeletal muscle fibrosis ( J:132154 )

• mutants treated with tamoxifen exhibit focal regions with increased fibrosis in skeletal muscle

impaired muscle relaxation ( J:132154 )

• mutants treated with tamoxifen to induce cre-recombination exhibit sustained myotonia, with waxing and waning myotonic runs noted on electromyogram

• mutants not treated with tamoxifen also develop myotonia, indicating basal leakiness of the transgene, however they do not show overt skeletal muscle wasting, histological abnormalities or muscle dysfunction as do tamoxifen treated mutants

skeleton

kyphosis ( J:132154 )

• by 4 weeks after tamoxifen administration, 70% of mutants show kyphosis

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
myotonic dystrophy type 1		DOID:11722	OMIM:160900	J:132154