All Phenotypes Tg(ACTA1-cre/Esr1*)2Kesr MGI Mouse

Phenotypes associated with this allele

Jump to	Allelic Composition	Genetic Background	Genotype ID
cn1	Gt(ROSA)26Sor^tm1(DUX4)Sqh/Gt(ROSA)26Sor⁺ Tg(ACTA1-cre/Esr1*)2Kesr/0	involves: 129S6/SvEvTac * C3H * C57BL/6	MGI:6280332
cn2	Bsg^tm1Itl/Bsg^tm1Itl Tg(ACTA1-cre/Esr1*)2Kesr/0	involves: 129S/SvEv * C3H * C57BL/6	MGI:6467231
cn3	Gt(ROSA)26Sor^{tm1.1(DUX4)Plj}/Gt(ROSA)26Sor⁺ Tg(ACTA1-cre/Esr1)2Kesr/0	involves: C3H * C57BL/6	MGI:6120564
cn4	Fam210a^{tm1c(EUCOMM)Wtsi}/Fam210a^{tm1c(EUCOMM)Wtsi} Tg(ACTA1-cre/Esr1*)2Kesr/0	involves: C3H * C57BL/6N * SJL	MGI:6159280

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

muscle

decreased skeletal muscle fiber diameter ( J:268959 )

• medium dose (5 mg/kg x 3 days/week) administration of tamoxifen by oral gavage results in smaller fiber diameters

centrally nucleated skeletal muscle fibers ( J:268959 )

• medium dose (5 mg/kg x 3 days/week) administration of tamoxifen by oral gavage results in diaphragms from 6 out of 18 mice that exhibit myofibers with greater than 10% central nuclei

skeletal muscle fiber degeneration ( J:268959 )

• high dose (150 mg/kg) administration of tamoxifen by oral gavage results in severe muscle degeneration in skeletal muscle within 7-9 days

• medium dose (5 mg/kg x 3 days/week) administration of tamoxifen by oral gavage results in damage to tibialis anterior, gastrocnemius, quadriceps and triceps within 4 weeks

• at 0.5mg/kg tamoxifen administration results in a decrease in mild sporadic lesions beginning at 1 month, with a dramatic increase in severity by 3 and 4 months

decreased skeletal muscle mass ( J:268959 )

• TA muscles in uninduced 1.5 year old mice are 33% smaller than controls with reduced absolute force output

muscle weakness ( J:268959 )

• medium dose (5 mg/kg x 3 days/week) administration of tamoxifen by oral gavage results in muscle weakness

• TA muscles in uninduced 1.5 year old mice develop reduced absolute force output

immune system

abnormal acute inflammation ( J:268959 )

• high dose (150 mg/kg) administration of tamoxifen by oral gavage results in immune cell infiltrates in skeletal muscle within 7-9 days

behavior/neurological

abnormal gait ( J:268959 )

• administration of tamoxifen (high dose - 150 mg/kg) by oral gavage results in a slow, unsteady gait, by 10 days mice are non-ambulatory

• administration of tamoxifen (medium dose - 5 mg/kg) by oral gavage results in gait changes within 3 weeks

decreased vertical activity ( J:268959 )

• medium dose (5 mg/kg x 3 days/week) administration of tamoxifen by oral gavage results in a reduction in rearing frequency

decreased locomotor activity ( J:268959 )

• high dose (150 mg/kg) administration of tamoxifen by oral gavage results in a reduction in overall activity, by 10 days mice are non-ambulatory

• medium dose (5 mg/kg x 3 days/week) administration of tamoxifen by oral gavage results in a reduction in total activity

• at 0.5mg/kg tamoxifen administration results in a decrease in activity beginning at 1 month, with significant reduction by 2 months, however mice recover to wild-type levels by 3-4 months

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
facioscapulohumeral muscular dystrophy		DOID:11727		J:268959

Allelic Composition	Bsg^tm1Itl/Bsg^tm1Itl Tg(ACTA1-cre/Esr1*)2Kesr/0
Genetic Background	involves: 129S/SvEv * C3H * C57BL/6

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Bsg^tm1Itl mutation (0 available); any Bsg mutation (15 available)
Tg(ACTA1-cre/Esr1*)2Kesr mutation (2 available)

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

muscle

muscle phenotype ( J:288233 )

N	• tamoxifen-treated mice exhibit normal exercise capacity and muscle ex vivo contractile properties

nervous system

nervous system phenotype ( J:288233 )

N	• tamoxifen-treated mice exhibit normal motorneurons

Allelic Composition	Gt(ROSA)26Sor^{tm1.1(DUX4)Plj}/Gt(ROSA)26Sor⁺ Tg(ACTA1-cre/Esr1)2Kesr/0
Genetic Background	involves: C3H * C57BL/6

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Gt(ROSA)26Sor^{tm1.1(DUX4*)Plj} mutation (1 available); any Gt(ROSA)26Sor mutation (944 available)
Tg(ACTA1-cre/Esr1*)2Kesr mutation (2 available)

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

behavior/neurological

ataxia ( J:256652 )

• mice exhibit ataxia within 7-8 days following IP injection of TMX

decreased grip strength ( J:256652 )

• mice exhibit loss of strength (hanging test) within 7-8 days following IP injection of TMX

muscle

skeletal muscle necrosis ( J:256652 )

• necrosis and phagocytosis are observed at time of euthanasia (9 days post TMX)

decreased skeletal muscle fiber size ( J:256652 )

• muscle from untreated mice has small irregular fibers

increased variability of skeletal muscle fiber size ( J:256652 )

• muscle from untreated mice has small irregular fibers

• heterogeneous fiber size distribution at time of euthanasia (9 days post TMX)

centrally nucleated skeletal muscle fibers ( J:256652 )

• muscle from untreated mice has patches of centralized nuclei

skeletal muscle fibrosis ( J:256652 )

• skeletal muscle tissues are mildly fibrotic at time of euthanasia (9 days post TMX)

dystrophic muscle ( J:256652 )

myopathy ( J:256652 )

• Tamoxifen (TMX)-treated mice exhibit progressive myopathy

• untreated mice exhibit a mild muscle phenotype

growth/size/body

weight loss ( J:256652 )

• mice exhibit weight loss within 7-8 days following IP injection of TMX

immune system

increased acute inflammation ( J:256652 )

• skeletal muscle tissues exhibit large infiltrates of mononuclear cells and loss of membrane integrity at time of euthanasia (9 days post TMX)

cellular

skeletal muscle necrosis ( J:256652 )

• necrosis and phagocytosis are observed at time of euthanasia (9 days post TMX)

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
facioscapulohumeral muscular dystrophy		DOID:11727		J:256652

Allelic Composition	Fam210a^{tm1c(EUCOMM)Wtsi}/Fam210a^{tm1c(EUCOMM)Wtsi} Tg(ACTA1-cre/Esr1*)2Kesr/0
Genetic Background	involves: C3H * C57BL/6N * SJL

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Fam210a^{tm1c(EUCOMM)Wtsi} mutation (1 available); any Fam210a mutation (17 available)
Tg(ACTA1-cre/Esr1*)2Kesr mutation (2 available)

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

skeleton

increased osteoclast cell number ( J:261817 )

• in tamoxifen-treated mice

decreased bone mineral density ( J:261817 )

• in tamoxifen-treated male mice

behavior/neurological

decreased grip strength ( J:261817 )

• in tamoxifen-treated mice

limbs/digits/tail

abnormal limb morphology ( J:261817 )

• decreased lean muscle mass in all limbs of tamoxifen-treated mice

growth/size/body

growth/size/body region phenotype ( J:261817 )

N	• tamoxifen-treated mice exhibit normal body weight

immune system

increased osteoclast cell number ( J:261817 )

• in tamoxifen-treated mice

hematopoietic system

increased osteoclast cell number ( J:261817 )

• in tamoxifen-treated mice

Contributing Projects: Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Models of Human Cancer database (MMHCdb) (formerly Mouse Tumor Biology (MTB)), Gene Ontology (GO)
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