All Phenotypes Isl1<tm1(cre)Tmj> MGI Mouse

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Phenotypes associated with this allele

Allele Symbol
Allele Name
Allele ID

Isl1^tm1(cre)Tmj
targeted mutation 1, Thomas M Jessell
MGI:2447758

Summary

21 genotypes

Jump to	Allelic Composition	Genetic Background	Genotype ID
cn1	Hand2^tm1Dsr/Hand2^tm2.1Dsr Isl1^tm1(cre)Tmj/Isl1⁺	involves: 129 * 129S7/SvEvBrd * 129X1/SvJ * C57BL/6	MGI:4940090
cn2	Isl1^tm1(cre)Tmj/Isl1⁺ Zfp503^tm1Lif/Zfp503^tm1Lif Tg(CAG-cat,-EGFP)1Rbns/0	involves: 129 * 129X1/SvJ * C57BL/6 * C57BL/6J	MGI:7606915
cn3	Isl1^tm1(cre)Tmj/Isl1⁺ Mapt^{tm1(Ewsr1/Etv4)Arbr}/Mapt⁺	involves: 129P2/OlaHsd * 129S1/Sv * 129X1/SvJ * C57BL/6	MGI:3716103
cn4	Nrg1^tm1Cbm/Nrg1^tm3Cbm Isl1^tm1(cre)Tmj/Isl1⁺	involves: 129P2/OlaHsd * 129X1/SvJ	MGI:2653523
cn5	Casz1^tm1.1Flc/Casz1^tm1.1Flc Isl1^tm1(cre)Tmj/Isl1⁺	involves: 129P2/OlaHsd * 129X1/SvJ * C57BL/6 * C57BL/6J * SJL	MGI:5811826
cn6	Maf^tm1.1Cbm/Maf^tm2.1Cbm Isl1^tm1(cre)Tmj/Isl1⁺	involves: 129P2/OlaHsd * 129X1/SvJ * C57BL/6 * SJL	MGI:5316895
cn7	Isl1^tm1(cre)Tmj/Isl1⁺ Shc1^tm9Paw/Shc1^tm9.1Paw	involves: 129S1/Sv * 129X1/SvJ	MGI:3717101
cn8	Dll4^tm1Frad/Dll4⁺ Isl1^tm1(cre)Tmj/Isl1⁺	involves: 129S1/Sv * 129X1/SvJ	MGI:7545577
cn9	Isl1^tm1(cre)Tmj/Isl1⁺ Shc1^tm9Paw/Shc1^tm9Paw	involves: 129S1/Sv * 129X1/SvJ	MGI:3717100
cn10	Brsk2^tm2.1Jrs/Brsk2^tm2.1Jrs Isl1^tm1(cre)Tmj/Isl1⁺	involves: 129S1/Sv * 129X1/SvJ * C57BL/6 * SJL	MGI:5524148
cn11	Isl1^tm1(cre)Tmj/Isl1⁺ Phox2b^tm3.1Jbr/Phox2b^tm3.1Jbr	involves: 129S2/SvPas * 129X1/SvJ	MGI:4438214
cn12	Phox2b^tm3.1Jbr/Phox2b^tm3.1Jbr Isl1^tm1(cre)Tmj/Isl1⁺	involves: 129S2/SvPas * 129X1/SvJ * C57BL/6 * DBA/2	MGI:4418306
cn13	Hand2^tm1Dsr/Hand2^tm2.1Dsr Gt(ROSA)26Sor^tm1Sor/Gt(ROSA)26Sor⁺ Isl1^tm1(cre)Tmj/Isl1⁺	involves: 129S4/SvJaeSor * 129S7/SvEvBrd * 129X1/SvJ * C57BL/6	MGI:4940091
cn14	Stk11^tm1.1Rdp/Stk11^tm1.1Rdp Isl1^tm1(cre)Tmj/Isl1⁺	involves: 129S6/SvEvTac * 129X1/SvJ	MGI:5524150
cn15	Smo^tm2Amc/Smo^tm2Amc Isl1^tm1(cre)Tmj/Isl1⁺	involves: 129S/Sv * 129X1/SvJ	MGI:5563905
cn16	Aldh1a2^tm1Soc/Aldh1a2^tm2Soc Isl1^tm1(cre)Tmj/Isl1⁺	involves: 129X1/SvJ	MGI:3665269
cn17	Etv1^tm1Wds/Etv1^tm1.1Wds Isl1^tm1(cre)Tmj/Isl1⁺	involves: 129X1/SvJ	MGI:3720888
cn18	Gt(ROSA)26Sor^{tm1(RARA)Soc}/Gt(ROSA)26Sor^{tm1(RARA)Soc} Isl1^tm1(cre)Tmj/Isl1⁺	involves: 129X1/SvJ	MGI:3795699
cn19	Rspo3^tm1Arte/Rspo3^tm1Arte Isl1^tm1(cre)Tmj/Isl1⁺	involves: 129X1/SvJ * C57BL/6	MGI:5643692
cn20	Nkx2-5^tm1Krc/Nkx2-5^tm1Krc Isl1^tm1(cre)Tmj/Isl1⁺	involves: 129X1/SvJ * C57BL/6	MGI:5643689
cn21	Isl1^tm1(cre)Tmj/Isl1⁺ Tg(SOD1*G37R)1Dwc/0	involves: 129X1/SvJ * C57BL/6	MGI:3629232

Allelic Composition	Hand2^tm1Dsr/Hand2^tm2.1Dsr Isl1^tm1(cre)Tmj/Isl1⁺
Genetic Background	involves: 129 * 129S7/SvEvBrd * 129X1/SvJ * C57BL/6

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Hand2^tm1Dsr mutation (0 available); any Hand2 mutation (12 available)
Hand2^tm2.1Dsr mutation (0 available); any Hand2 mutation (12 available)
Isl1^tm1(cre)Tmj mutation (0 available); any Isl1 mutation (33 available)

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

mortality/aging

embryonic lethality during organogenesis, complete penetrance ( J:169213 )

• mice die at E10.5

cardiovascular system

abnormal cardiac outflow tract development ( J:169213 )

• shortened

heart right ventricle hypoplasia ( J:169213 )

• severe

cellular

increased apoptosis ( J:169213 )

• at E9.0, mice exhibit increased apoptosis in the pharyngeal mesoderm compared with wild-type mice

Allelic Composition	Isl1^tm1(cre)Tmj/Isl1⁺ Zfp503^tm1Lif/Zfp503^tm1Lif Tg(CAG-cat,-EGFP)1Rbns/0
Genetic Background	involves: 129 * 129X1/SvJ * C57BL/6 * C57BL/6J

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Isl1^tm1(cre)Tmj mutation (0 available); any Isl1 mutation (33 available)
Tg(CAG-cat,-EGFP)1Rbns mutation (1 available)
Zfp503^tm1Lif mutation (0 available); any Zfp503 mutation (36 available)

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

nervous system

abnormal striatum morphology ( J:286635 )

• marked reduction in striatonigral GFP+ axons along routes of striatonigral axonal projections

abnormal dorsal striatum morphology ( J:286635 )

• smaller at E18.5

abnormal ventral striatum morphology ( J:286635 )

• larger at E18.5

Allelic Composition	Isl1^tm1(cre)Tmj/Isl1⁺ Mapt^{tm1(Ewsr1/Etv4)Arbr}/Mapt⁺
Genetic Background	involves: 129P2/OlaHsd * 129S1/Sv * 129X1/SvJ * C57BL/6

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Isl1^tm1(cre)Tmj mutation (0 available); any Isl1 mutation (33 available)
Mapt^{tm1(Ewsr1/Etv4)Arbr} mutation (0 available); any Mapt mutation (428 available)

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

nervous system

decreased muscle spindle number ( J:100886 )

• mice have 25% of the wild-type number of muscle spindles

abnormal sensory neuron morphology ( J:100886 )

• while sensory axons reach the skin only rudimentary sensory axon branching is established within the skin

abnormal sensory neuron innervation pattern ( J:100886 )

• sensory afferents fail to invade the spinal cord and are found in an extreme lateral position at the dorsal root entry zone

• sensory afferents are bifurcated at the entry point

• sensory afferent fibers fail to approach the midline at the distal segments and continue to occupy an extreme lateral position

abnormal dorsal root ganglion morphology ( J:100886 )

• unlike wild-type cells, dorsal root ganglia neurons survive in culture without the addition of neurotrophic agents

• whole dorsal root ganglia require neurotrophin-3 for survival

muscle

decreased muscle spindle number ( J:100886 )

• mice have 25% of the wild-type number of muscle spindles

Allelic Composition	Nrg1^tm1Cbm/Nrg1^tm3Cbm Isl1^tm1(cre)Tmj/Isl1⁺
Genetic Background	involves: 129P2/OlaHsd * 129X1/SvJ

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Isl1^tm1(cre)Tmj mutation (0 available); any Isl1 mutation (33 available)
Nrg1^tm1Cbm mutation (0 available); any Nrg1 mutation (53 available)
Nrg1^tm3Cbm mutation (0 available); any Nrg1 mutation (53 available)

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

muscle

abnormal muscle spindle morphology ( J:80793 )

• mutants exhibit a defect in muscle spindle differentiation in the dorsal root ganglion and motor neurons

nervous system

abnormal muscle spindle morphology ( J:80793 )

• mutants exhibit a defect in muscle spindle differentiation in the dorsal root ganglion and motor neurons

decreased Schwann cell number ( J:80793 )

• selective absence of Schwann cells at E16.5 in adductor and gracilis muscles but not in other muscles

abnormal proprioceptive neuron morphology ( J:80793 )

• parvalbumin+ proprioceptive afferents are present in E16.5 hindlimb muscles and initiate contact with individual myofibers, but they do not develop annulospiral branches around the myofibers

• parvalbumin+ proprioceptive terminals at muscle spindles remain primitive and unbranched at E18.5

• however, survival and initial differentiation of proprioceptive afferent sensory neurons is not impaired

Allelic Composition	Casz1^tm1.1Flc/Casz1^tm1.1Flc Isl1^tm1(cre)Tmj/Isl1⁺
Genetic Background	involves: 129P2/OlaHsd * 129X1/SvJ * C57BL/6 * C57BL/6J * SJL

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Casz1^tm1.1Flc mutation (1 available); any Casz1 mutation (344 available)
Isl1^tm1(cre)Tmj mutation (0 available); any Isl1 mutation (33 available)

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

mortality/aging

lethality throughout fetal growth and development, complete penetrance ( J:221324 )

• embryos are viable and indistinguishable from controls at least until E14.5; however, no mice are recovered postnatally

cardiovascular system

abnormal heart shape ( J:221324 )

• misshapen heart at E14.5

• however, no ventricular septal defects are detected at E14.5

abnormal heart right ventricle morphology ( J:221324 )

• right ventricular wall thickness is significantly reduced at E14.5

• however, left ventricular wall thickness is normal

heart right ventricle hypoplasia ( J:221324 )

• right ventricular hypoplasia at E14.5

cellular

decreased mitotic index ( J:221324 )

• at E12.5, cardiomyocyte mitotic index is significantly reduced in the right ventricles (but not in the left ventricles), as shown by phospho-histone H3 staining

Allelic Composition	Maf^tm1.1Cbm/Maf^tm2.1Cbm Isl1^tm1(cre)Tmj/Isl1⁺
Genetic Background	involves: 129P2/OlaHsd * 129X1/SvJ * C57BL/6 * SJL

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Isl1^tm1(cre)Tmj mutation (0 available); any Isl1 mutation (33 available)
Maf^tm1.1Cbm mutation (0 available); any Maf mutation (26 available)
Maf^tm2.1Cbm mutation (0 available); any Maf mutation (26 available)

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

nervous system

abnormal axon morphology ( J:181639 )

• large diameter axons in the saphenous nerve become thinner

• many myelinated axons greater than 4 um are lost in the interosseous nerve

abnormal Meissner's corpuscle morphology ( J:181639 )

• reduced in number and rudimentary

• however, NF200+ axons innervating the dermal papillae are normal

abnormal pacinian corpuscle morphology ( J:181639 )

• reduced in number with axonal loss

• remaining corpuscles are small and have irregularly shaped cores

decreased pacinian corpuscle number ( J:181639 )

small pacinian corpuscles ( J:181639 )

axon degeneration ( J:181639 )

• innervating Pacinian corpuscles

abnormal nervous system electrophysiology ( J:181639 )

• following skin indentation, mice exhibit prolonged rapid adapting mechanoreceptor (RAM) firing that continues into the beginning of the static phase with increased RAM spiking over a wide range of displacement amplitudes and shortened spike intervals compared with control mice

• following skin indentation, mice exhibit decreased von Frey thresholds of short adapting mechanoreceptors compared with control mice

• mice treated with linopirdine fail to exhibit a decrease in interspike interval in the saphenous nerve unlike control mice

• however, mice exhibit normal short adapting mechanoreceptor, D-hair receptors and Adelta nociceptor firing following skin indentation

decreased nerve conduction velocity ( J:181639 )

• in large diameter but not medium diameter fibers

integument

abnormal coat/ hair morphology ( J:181639 )

• the hair in the dorsal hindpaw is shorter than the hair in the back skin and resembles hair of the tail

growth/size/body

decreased body weight ( J:181639 )

• mild in adults

• however, mice exhibit normal body weight at P15

behavior/neurological

impaired coordination ( J:181639 )

• on a rotarod test

Allelic Composition	Isl1^tm1(cre)Tmj/Isl1⁺ Shc1^tm9Paw/Shc1^tm9.1Paw
Genetic Background	involves: 129S1/Sv * 129X1/SvJ

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Isl1^tm1(cre)Tmj mutation (0 available); any Isl1 mutation (33 available)
Shc1^tm9.1Paw mutation (0 available); any Shc1 mutation (66 available)
Shc1^tm9Paw mutation (0 available); any Shc1 mutation (66 available)

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

normal phenotype

no abnormal phenotype detected ( J:122927 )

• mutants appear normal

Allelic Composition	Dll4^tm1Frad/Dll4⁺ Isl1^tm1(cre)Tmj/Isl1⁺
Genetic Background	involves: 129S1/Sv * 129X1/SvJ

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Dll4^tm1Frad mutation (0 available); any Dll4 mutation (26 available)
Isl1^tm1(cre)Tmj mutation (0 available); any Isl1 mutation (33 available)

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

cardiovascular system

abnormal cardiac outflow tract development ( J:308916 )

• 9 of 28 embryos show outflow tract alignment defects

• however, septation of the outflow tract is preserved

double outlet right ventricle ( J:308916 )

• in embryos with a severe ventricular septal defect

overriding aortic valve ( J:308916 )

• in embryos with a shallower ventricular septal defect

ventricular septal defect ( J:308916 )

• some embryos show a prominent ventricular septal defect while in others this is more shallow

abnormal pulmonary valve morphology ( J:308916 )

• pulmonary valve arises more cranially and exits the right ventricle

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
Adams-Oliver syndrome		DOID:0060227	OMIM:100300 OMIM:614219 OMIM:614814 OMIM:615297 OMIM:616028 OMIM:PS100300	J:308916

Allelic Composition	Isl1^tm1(cre)Tmj/Isl1⁺ Shc1^tm9Paw/Shc1^tm9Paw
Genetic Background	involves: 129S1/Sv * 129X1/SvJ

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Isl1^tm1(cre)Tmj mutation (0 available); any Isl1 mutation (33 available)
Shc1^tm9Paw mutation (0 available); any Shc1 mutation (66 available)

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

normal phenotype

no abnormal phenotype detected ( J:122927 )

• mutants appear normal

Allelic Composition	Brsk2^tm2.1Jrs/Brsk2^tm2.1Jrs Isl1^tm1(cre)Tmj/Isl1⁺
Genetic Background	involves: 129S1/Sv * 129X1/SvJ * C57BL/6 * SJL

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Brsk2^tm2.1Jrs mutation (1 available); any Brsk2 mutation (37 available)
Isl1^tm1(cre)Tmj mutation (0 available); any Isl1 mutation (33 available)

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

mortality/aging

neonatal lethality, incomplete penetrance ( J:201695 )

• few mice survive beyond 24 hours after birth

nervous system

nervous system phenotype ( J:201695 )

N	• interneurons exhibit normal migration to proper positions Golgi tendon organs are normally innervated

abnormal axon extension ( J:201695 )

• reduced outgrowths from type Ia proprioceptive sensory neuron in dorsal root ganglia explants in the presence of NT-3 or NGF

abnormal sensory neuron innervation pattern ( J:201695 )

• type Ia proprioceptive sensory neuron projections are disrupted and exhibit arrest at E15.5 as they reach the brainstem or ventral spinal cord in the medial spinal cord adjacent to the central canal and entire rostral-caudal extent of the spinal cord that persists through fetal development until P8 compared to in control mice

• type Ia proprioceptive sensory neuron projections fail to form terminal arbors

• mice exhibit defects in a second population of sensory neurons with few axons of the T12 dorsal root ganglion that cross the midline and only rare occurrences of axons that reach the proper contralateral target compared with control mice

• whisker axons have sparse arbors that fail to reach the correct target region in the bed nucleus of the stria terminalis (BSTC) without extensive branching

• however, mice exhibit normal growth of the sensory axons in the spinal cord and neuron targeting of nociceptive sensory axons in laminae I/IIi/IIo

• however, mice exhibit normal Pv+ proprioceptive axons growth into fore and hind limb muscles, trunk sensory axons form normal disc shaped endings on Merkel cells in the epidermis and axons in the deep vibrissal nerve that innervate whisker follicles

abnormal proprioceptive neuron morphology ( J:201695 )

• type Ia proprioceptive sensory neuron projections fail to form terminal arbors

• fewer proprioceptor axons in the cuneate nucleus, but not the cuneate fascicle

• however, growth of the sensory axons in the spinal cord is normal

behavior/neurological

absent gastric milk in neonates ( J:201695 )

• little milk in stomachs

bradykinesia ( J:201695 )

• hypokinetic

cellular

abnormal axon extension ( J:201695 )

• reduced outgrowths from type Ia proprioceptive sensory neuron in dorsal root ganglia explants in the presence of NT-3 or NGF

Allelic Composition	Isl1^tm1(cre)Tmj/Isl1⁺ Phox2b^tm3.1Jbr/Phox2b^tm3.1Jbr
Genetic Background	involves: 129S2/SvPas * 129X1/SvJ

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Isl1^tm1(cre)Tmj mutation (0 available); any Isl1 mutation (33 available)
Phox2b^tm3.1Jbr mutation (2 available); any Phox2b mutation (25 available)

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

nervous system

abnormal pterygopalatine ganglion morphology ( J:157532 )

• fails to form, on six sides out of eight, in four embryos

absent petrosal ganglion ( J:157532 )

• missing, on five sides out of six, in three embryos

Allelic Composition	Phox2b^tm3.1Jbr/Phox2b^tm3.1Jbr Isl1^tm1(cre)Tmj/Isl1⁺
Genetic Background	involves: 129S2/SvPas * 129X1/SvJ * C57BL/6 * DBA/2

Find Mice

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

mortality/aging

postnatal lethality, complete penetrance ( J:155885 )

• 1 week after birth, few mice are still alive

nervous system

abnormal neuron differentiation ( J:155885 )

• mice exhibit abnormal development of facial neuron precursors that do not migrate into r6 unlike in wild-type mice

• mice exhibit impaired development of retrotapezoid nucleus neurons compared with wild-type mice

abnormal neuron physiology ( J:155885 )

• the parafacial area e-pF oscillator exhibits only occasional motor activity bursts with reduced frequency compared to in wild-type mice

• rhythmic phrenic discharges are less frequent than in wild-type mice

• mice fail to exhibit an accelerated respiratory-like rhythm phrenic discharge in response to low pH challenge unlike similarly treated wild-type mice

growth/size/body

decreased body size ( J:155885 )

• surviving mice are smaller than wild-type mice

cellular

abnormal neuron differentiation ( J:155885 )

• mice exhibit abnormal development of facial neuron precursors that do not migrate into r6 unlike in wild-type mice

• mice exhibit impaired development of retrotapezoid nucleus neurons compared with wild-type mice

Allelic Composition	Hand2^tm1Dsr/Hand2^tm2.1Dsr Gt(ROSA)26Sor^tm1Sor/Gt(ROSA)26Sor⁺ Isl1^tm1(cre)Tmj/Isl1⁺
Genetic Background	involves: 129S4/SvJaeSor * 129S7/SvEvBrd * 129X1/SvJ * C57BL/6

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Gt(ROSA)26Sor^tm1Sor mutation (8 available); any Gt(ROSA)26Sor mutation (942 available)
Hand2^tm1Dsr mutation (0 available); any Hand2 mutation (12 available)
Hand2^tm2.1Dsr mutation (0 available); any Hand2 mutation (12 available)
Isl1^tm1(cre)Tmj mutation (0 available); any Isl1 mutation (33 available)

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

cardiovascular system

abnormal heart development ( J:169213 )

• at E9.5, mice exhibit fewer progenitor cells migration into the outflow tract compared with control mice

Allelic Composition	Stk11^tm1.1Rdp/Stk11^tm1.1Rdp Isl1^tm1(cre)Tmj/Isl1⁺
Genetic Background	involves: 129S6/SvEvTac * 129X1/SvJ

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Isl1^tm1(cre)Tmj mutation (0 available); any Isl1 mutation (33 available)
Stk11^tm1.1Rdp mutation (0 available); any Stk11 mutation (34 available)

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

mortality/aging

decreased tumor-free survival time ( J:201695 )

• mice are euthanized at 4 to 6 weeks due to massive gastrointestinal tumors

• however, mice survive to birth and postnatally

neoplasm

increased gastrointestinal tumor incidence ( J:201695 )

• massive gastrointestinal tumors

digestive/alimentary system

increased gastrointestinal tumor incidence ( J:201695 )

• massive gastrointestinal tumors

nervous system

nervous system phenotype ( J:201695 )

• axon bundles are normal in the brainstem trigeminal complex, ascending tracts in the spinal cord (spinocerebellar, spinothalamic and dorsal funiculus), the optic nerve and motor and sensory nerves in the periphery

• mice exhibit normal type Ia proprioceptive sensory neuron projections to the ventral horn

• mice exhibit no sensory or motor deficit

abnormal cerebral cortex morphology ( J:201695 )

• axons tracts in the cortical intermediate zone are reduced compared to in control mice

thin cerebral cortex ( J:201695 )

• thin-walled

behavior/neurological

behavior/neurological phenotype ( J:201695 )

N	• mice exhibit no sensory or motor deficit

Allelic Composition	Smo^tm2Amc/Smo^tm2Amc Isl1^tm1(cre)Tmj/Isl1⁺
Genetic Background	involves: 129S/Sv * 129X1/SvJ

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Isl1^tm1(cre)Tmj mutation (0 available); any Isl1 mutation (33 available)
Smo^tm2Amc mutation (1 available); any Smo mutation (39 available)

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

cardiovascular system

blood vessel atresia ( J:204743 )

• embryos show severe inflow tract defects with pulmonary vein atresia

persistent truncus arteriosus ( J:204743 )

abnormal heart right ventricle morphology ( J:204743 )

• embryos show severe inflow tract defects with pulmonary vein atresia

abnormal blood circulation ( J:204743 )

• embryos show persistence of aortopulmonary collateral circulation

Allelic Composition	Aldh1a2^tm1Soc/Aldh1a2^tm2Soc Isl1^tm1(cre)Tmj/Isl1⁺
Genetic Background	involves: 129X1/SvJ

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Aldh1a2^tm1Soc mutation (0 available); any Aldh1a2 mutation (36 available)
Aldh1a2^tm2Soc mutation (0 available); any Aldh1a2 mutation (36 available)
Isl1^tm1(cre)Tmj mutation (0 available); any Isl1 mutation (33 available)

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

nervous system

abnormal motor neuron innervation pattern ( J:112635 )

• at E13.5 both ventral and dorsal projecting lateral motor column neurons appear stunted or atrophied and in some cases are retracted

decreased motor neuron number ( J:112635 )

• at E13.5 at the forelimb level the number of lateral motor column medial and lateral motor neurons are reduced by about 30% and 25%, respectively; however no difference in the number of lateral motor column lateral motor neurons is detected at E12.5

• at E13.5 at the hindlimb level the number of lateral motor column medial and lateral motor neurons are reduced by about 17% and 20%, respectively

Allelic Composition	Etv1^tm1Wds/Etv1^tm1.1Wds Isl1^tm1(cre)Tmj/Isl1⁺
Genetic Background	involves: 129X1/SvJ

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Etv1^tm1.1Wds mutation (0 available); any Etv1 mutation (44 available)
Etv1^tm1Wds mutation (2 available); any Etv1 mutation (44 available)
Isl1^tm1(cre)Tmj mutation (0 available); any Isl1 mutation (33 available)

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

nervous system

abnormal proprioceptive neuron morphology ( J:83461 )

• proprioceptive afferents terminate prematurely in the intrmediate zone of the spinal cord

Allelic Composition	Gt(ROSA)26Sor^{tm1(RARA)Soc}/Gt(ROSA)26Sor^{tm1(RARA)Soc} Isl1^tm1(cre)Tmj/Isl1⁺
Genetic Background	involves: 129X1/SvJ

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Gt(ROSA)26Sor^{tm1(RARA*)Soc} mutation (1 available); any Gt(ROSA)26Sor mutation (942 available)
Isl1^tm1(cre)Tmj mutation (0 available); any Isl1 mutation (33 available)

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

nervous system

decreased motor neuron number ( J:135403 )

• mice exhibit a 35% decrease in Lim1+/Islet2+ motor neurons compared to wild-type mice

Allelic Composition	Rspo3^tm1Arte/Rspo3^tm1Arte Isl1^tm1(cre)Tmj/Isl1⁺
Genetic Background	involves: 129X1/SvJ * C57BL/6

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Isl1^tm1(cre)Tmj mutation (0 available); any Isl1 mutation (33 available)
Rspo3^tm1Arte mutation (0 available); any Rspo3 mutation (14 available)

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

mortality/aging

lethality throughout fetal growth and development, complete penetrance ( J:214093 )

• late gestational lethality at E14.5-E16.5 in most mice due to cardiovascular failure

cardiovascular system

double outlet right ventricle ( J:214093 )

• all mice exhibit small right ventricle and/or double outlet right ventricle

decreased heart right ventricle size ( J:214093 )

• all mice exhibit small right ventricle and/or double outlet right ventricle

pericardial edema ( J:214093 )

homeostasis/metabolism

pericardial edema ( J:214093 )

Allelic Composition	Nkx2-5^tm1Krc/Nkx2-5^tm1Krc Isl1^tm1(cre)Tmj/Isl1⁺
Genetic Background	involves: 129X1/SvJ * C57BL/6

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Isl1^tm1(cre)Tmj mutation (0 available); any Isl1 mutation (33 available)
Nkx2-5^tm1Krc mutation (0 available); any Nkx2-5 mutation (21 available)

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

mortality/aging

perinatal lethality ( J:214093 )

• cyanosis and death within a few hours of birth; perinatal lethality is due to outflow tract septation defects

cardiovascular system

abnormal myocardial trabeculae morphology ( J:214093 )

• the trabecular myocardium, but not the compact myocardium, shows a reduction in the relative proliferation rate in embryos

abnormal cardiac outflow tract development ( J:214093 )

• foreshortened outflow tract

• complete penetrance of outflow tract defects

• the proximal outflow tract shows defective endocardial networks, while the neural crest-derived distal outflow tract endocardial cushions appear normal

abnormal conotruncal ridge morphology ( J:214093 )

• smaller developing endocardial cushions in the proximal outflow tract

• the outflow tract cushions show a reduction in the relative proliferation rate in embryos

persistent truncus arteriosus ( J:214093 )

• 92% of mutants exhibit a single outflow tract arising from the right ventricle

double outlet right ventricle ( J:214093 )

• only a small number of embryos show double outlet right ventricle

abnormal heart right ventricle morphology ( J:214093 )

• reduction of trabecular networks in the right ventricle, whereas the left ventricle is normal

decreased heart right ventricle size ( J:214093 )

heart right ventricle hypoplasia ( J:214093 )

• decrease in the relative proliferative rate of the second heart field is evident as early as E10, resulting in hypoplastic right ventricle at E12.5

homeostasis/metabolism

cyanosis ( J:214093 )

muscle

abnormal myocardial trabeculae morphology ( J:214093 )

• the trabecular myocardium, but not the compact myocardium, shows a reduction in the relative proliferation rate in embryos

Allelic Composition	Isl1^tm1(cre)Tmj/Isl1⁺ Tg(SOD1*G37R)1Dwc/0
Genetic Background	involves: 129X1/SvJ * C57BL/6

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Isl1^tm1(cre)Tmj mutation (0 available); any Isl1 mutation (33 available)
Tg(SOD1*G37R)1Dwc mutation (1 available)

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

mortality/aging

premature death ( J:109131 )

• overall survival is extended 64 days

nervous system

axon degeneration ( J:109131 )

• in transgenic mice expressing a motorneuron specific Cre, disease onset is delayed 18 days

• progression from onset through early disease is delayed 31 days

• later disease progression is slowed with an average extension of 15 days

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
amyotrophic lateral sclerosis type 1		DOID:0060193	OMIM:105400	J:109131

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