Phenotypes associated with this allele

• Allele Symbol: Tg(Prrx1-cre/ERT2)1Mlgn
• Allele Name: transgene insertion 1, Malcolm P Logan
• Allele ID: MGI:4437919
• Summary: 5 genotypes

Jump to	Allelic Composition	Genetic Background	Genotype ID
cn1	Tbx4^tm1.2Pa/Tbx4^tm1.2Pa Tg(Prrx1-cre/ERT2)1Mlgn/0	involves: 129S1/Sv * 129X1/SvJ	MGI:4442421
cn2	Gt(ROSA)26Sor^tm5(ASPSCR1/TFE3)Mrc/Gt(ROSA)26Sor^+ Tg(Prrx1-cre/ERT2)1Mlgn/0	involves: 129S1/Sv * 129X1/SvJ * C57BL/6	MGI:5649279
cn3	Gt(ROSA)26Sor^tm4(EWSR1/ATF1)Mrc/Gt(ROSA)26Sor^+ Tg(Prrx1-cre/ERT2)1Mlgn/?	involves: C57BL/6	MGI:5495320
cn4	Tbx5^tm1Jse/Tbx5^tm1Jse Tg(Prrx1-cre/ERT2)1Mlgn/0 Tg(Scx-GFP)1Stzr/0	Not Specified	MGI:4442422
cn5	Tbx5^tm1Jse/Tbx5^tm1Jse Tg(Prrx1-cre/ERT2)1Mlgn/0	Not Specified	MGI:4442420

Genotype
MGI:4442421

cn1

• Allelic Composition: Tbx4^tm1.2Pa/Tbx4^tm1.2Pa; Tg(Prrx1-cre/ERT2)1Mlgn/0
• Genetic Background: involves: 129S1/Sv * 129X1/SvJ

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

limbs/digits/tail

abnormal hindlimb morphology ( J:157917 )

• at E16.5 in contrast to wild-type hindlimbs, in the analogous regions to the musculus lumbricales (Lum) and m flexor digitorum brevis (fdb) in mutant limbs, muscles misinsert and display the wrong shape with tamoxifen treatment at E10.5

• all limb musculature is affected

• limb muscle formation is unaffected if tamoxifen administration is done at E12.5

• terminal differentiation of limb muscle is not affected

abnormal limb development ( J:157917 )

Genotype
MGI:5649279

cn2

• Allelic Composition: Gt(ROSA)26Sor^{tm5(ASPSCR1/TFE3)Mrc}/Gt(ROSA)26Sor⁺; Tg(Prrx1-cre/ERT2)1Mlgn/0
• Genetic Background: involves: 129S1/Sv * 129X1/SvJ * C57BL/6

neoplasm

increased organ/body region tumor incidence ( J:217462 )

• mice develop intracranial tumors 10-14 weeks after tamoxifen treatment at 2 weeks of age

• tumors are located either within the brain parenchyma or it the periosteum along the inner table of the cranium

• however, no tumors are seen in the outer table periosteum or the limbs

Genotype
MGI:5495320

cn3

• Allelic Composition: Gt(ROSA)26Sor^{tm4(EWSR1/ATF1)Mrc}/Gt(ROSA)26Sor⁺; Tg(Prrx1-cre/ERT2)1Mlgn/?
• Genetic Background: involves: C57BL/6

muscle

myopathy ( J:194308 )

• when tamoxifen is administered after 3 weeks of age myopathy develops by 12 months

neoplasm

increased tumor incidence ( J:194308 )

• when tamoxifen is administered after 3 weeks of age tumors develop in the extremities of 100% of mice by 8 weeks of age

• tumors arise from the periosteal/perichondrial layer as well as within musculature

• clear cell morphology or spindle cell morphology

Genotype
MGI:4442422

cn4

• Allelic Composition: Tbx5^tm1Jse/Tbx5^tm1Jse; Tg(Prrx1-cre/ERT2)1Mlgn/0; Tg(Scx-GFP)1Stzr/0
• Genetic Background: Not Specified

muscle

abnormal tendon morphology ( J:157917 )

• some tendon fibers connecting forearm muscles to skeletal elements in the handplate are thinner than in controls and some have fused together

• in forelimb, tendons develop functional entheses on the forming skeleton

limbs/digits/tail

abnormal forelimb morphology ( J:157917 )

• with tamoxifen treatment at E9.5, limbs display minor skeletal deformities similar to those in humans with Holt-Oram syndrome (triphalangeal thumb)

• muscle patterning is disrupted similarly to that with tamoxifen treatment at E10.5

• patterning of tendon fibers connecting forearm muscles to skeletal elements in the handplate is significantly disrupted compared to wild-type; fewer tendon fibers are present

• mispatterned muscles make functional myotendinous attachments to tendons

abnormal limb development ( J:157917 )

skeleton

abnormal tendon morphology ( J:157917 )

• some tendon fibers connecting forearm muscles to skeletal elements in the handplate are thinner than in controls and some have fused together

• in forelimb, tendons develop functional entheses on the forming skeleton

Genotype
MGI:4442420

cn5

• Allelic Composition: Tbx5^tm1Jse/Tbx5^tm1Jse; Tg(Prrx1-cre/ERT2)1Mlgn/0
• Genetic Background: Not Specified

limbs/digits/tail

abnormal forelimb morphology ( J:157917 )

• at E16.5 in contrast to wild-type forelimbs where the spinodeltoidus (Spd), musculus triceps brachii longus (Tbl), and m triceps brachii lateralis (Tblt) muscles have specific origin and insertion sites, muscle bundles in the equivalent region of mutant limbs show a common origin at one focus and have split to form smaller muscle bundles that insert at aberrant sites when tamoxifen treatment is performed at E10.5; mispatterning of emerging muscles and ectopic splitting of nascent muscle bundles in observed by E13.5 with tamoxifen treatment at E10.5

• with tamoxifen treatment at E10.5, in the forelimb the m triceps brachii medialis (Tbm), Tbl, and Tblt, which insert on the olecranon process of the ulna in wild-type, show ectopic splitting into additional bundles which insert in the more distal shaft of the ulna

• zeugopodal muscles such as the m extensor digitorum communis (Edc) are ectopically split

• all limb musculature is affected

• limb muscle formation is unaffected if tamoxifen administration is done at E11.5

• terminal differentiation of limb muscle is not affected

abnormal limb development ( J:157917 )