Phenotypes associated with this allele

• Allele Symbol: Tg(GFAP-cre)#Gtm
• Allele Name: transgene insertion #, David H Gutmann
• Allele ID: MGI:3057344
• Summary: 12 genotypes

Jump to	Allelic Composition	Genetic Background	Genotype ID
cn1	Smarcb1^tm2Sho/Smarcb1^tm2Sho Trp53^tm1Brn/Trp53^+ Tg(GFAP-cre)#Gtm/0	involves: 129P2/OlaHsd * 129S1/Sv * C57BL/6 * CBA	MGI:5771804
cn2	Smarcb1^tm2Sho/Smarcb1^+ Trp53^tm1Brn/Trp53^+ Tg(GFAP-cre)#Gtm/0	involves: 129P2/OlaHsd * 129S1/Sv * C57BL/6 * CBA	MGI:5771802
cn3	Smarcb1^tm2Sho/Smarcb1^+ Trp53^tm1Brn/Trp53^tm1Brn Tg(GFAP-cre)#Gtm/0	involves: 129P2/OlaHsd * 129S1/Sv * C57BL/6 * CBA	MGI:5771803
cn4	Smarcb1^tm2Sho/Smarcb1^tm2Sho Trp53^tm1Brn/Trp53^tm1Brn Tg(GFAP-cre)#Gtm/0	involves: 129P2/OlaHsd * 129S1/Sv * C57BL/6 * CBA	MGI:5771805
cn5	Itgav^tm2Hyn/Itgav^tm2.1Hyn Tg(GFAP-cre)#Gtm/0	involves: 129P2/OlaHsd * C57BL/6 * CBA	MGI:5306155
cn6	Smarcb1^tm2Sho/Smarcb1^tm2Sho Tg(GFAP-cre)#Gtm/0	involves: 129S1/Sv * C57BL/6 * CBA	MGI:5771801
cn7	Tsc1^tm1Djk/Tsc1^tm1Djk Tg(GFAP-cre)#Gtm/0	involves: 129S4/SvJae * C57BL/6 * CBA	MGI:5292549
cn8	Gt(ROSA)26Sor^tm2(tTA,CMV*1-KIAA1549/BRAF,-EGFP)Gtm/Gt(ROSA)26Sor^+ Tg(GFAP-cre)#Gtm/0	involves: 129S6/SvEvTac * C57BL/6 * CBA	MGI:5543816
cn9	Nf1^tm1Par/Nf1^tm1Fcr Tg(GFAP-cre)#Gtm/0	involves: 129S/SvEv * 129S1/Sv * 129X1/SvJ * C57BL/6 * CBA	MGI:4838320
cn10	Nf1^tm1Fcr/Nf1^+ Tsc1^tm1Djk/Tsc1^tm1Djk Tg(GFAP-cre)#Gtm/0	involves: 129S/SvEv * 129S4/SvJae * C57BL/6 * CBA	MGI:5292550
cn11	Nf1^tm1Fcr/Nf1^+ Tg(GFAP-cre)#Gtm/0	involves: 129S/SvEv * 129S4/SvJae * C57BL/6 * CBA	MGI:5292552
cn12	Gt(ROSA)26Sor^tm1(tTA,CMV*1-Rheb,-EGFP)Gtm/Gt(ROSA)26Sor^+ Tg(GFAP-cre)#Gtm/0	involves: C57BL/6 * CBA	MGI:5292547

Genotype
MGI:5771804

cn1

• Allelic Composition: Smarcb1^tm2Sho/Smarcb1^tm2Sho; Trp53^tm1Brn/Trp53⁺; Tg(GFAP-cre)#Gtm/0
• Genetic Background: involves: 129P2/OlaHsd * 129S1/Sv * C57BL/6 * CBA

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

nervous system

abnormal brain white matter morphology ( J:226786 )

• lesions in the white matter of the corpus callosum and cingulum

neoplasm

neoplasm ( J:226786 )

N • no neoplastic growth is seen in the brain

Genotype
MGI:5771802

cn2

• Allelic Composition: Smarcb1^tm2Sho/Smarcb1⁺; Trp53^tm1Brn/Trp53⁺; Tg(GFAP-cre)#Gtm/0
• Genetic Background: involves: 129P2/OlaHsd * 129S1/Sv * C57BL/6 * CBA

behavior/neurological

excessive scratching ( J:226786 )

• excessive scratching behavior resulting in dermatitis around the neck, earlobes, and the flank region

Genotype
MGI:5771803

cn3

• Allelic Composition: Smarcb1^tm2Sho/Smarcb1⁺; Trp53^tm1Brn/Trp53^tm1Brn; Tg(GFAP-cre)#Gtm/0
• Genetic Background: involves: 129P2/OlaHsd * 129S1/Sv * C57BL/6 * CBA

behavior/neurological

excessive scratching ( J:226786 )

• excessive scratching behavior resulting in dermatitis around the neck, earlobes, and the flank region

Genotype
MGI:5771805

cn4

• Allelic Composition: Smarcb1^tm2Sho/Smarcb1^tm2Sho; Trp53^tm1Brn/Trp53^tm1Brn; Tg(GFAP-cre)#Gtm/0
• Genetic Background: involves: 129P2/OlaHsd * 129S1/Sv * C57BL/6 * CBA

behavior/neurological

lethargy ( J:226786 )

• many mice appear lethargic with hind limb paralysis

excessive scratching ( J:226786 )

• severe scratching behavior

dystonia ( J:226786 )

• mice exhibit intermittent episodes of tail extension and dystonic posturing of the body, frequently in response to handling

tremors ( J:226786 )

impaired coordination ( J:226786 )

• motor coordination problems become evident from around 3 weeks of age

abnormal limb posture ( J:226786 )

• abnormal posture of the hind limbs

abnormal locomotor behavior ( J:226786 )

• mice exhibit tumbling repeatedly during attempted locomotion; mice frequently fall over and repeatedly lift and replace the same paw in various positions during each stride

ataxia ( J:226786 )

• severe ataxia

abnormal gait ( J:226786 )

• mice exhibit a non-uniform gait, with uneven stride length and width, dragging of the hindpaws and an inability to keep the hindquarters upr

abnormal stride length ( J:226786 )

• uneven stride length

hindlimb paralysis ( J:226786 )

• many mice appear lethargic with hind limb paralysis

circling ( J:226786 )

seizures ( J:226786 )

• mice exhibit clear seizures or intermittent electrographic seizures associated either with forelimb clonus, evolving into body jerking, and wild running or arrest of activity during the ictal EEG discharges

cellular

abnormal cerebellar granule cell migration ( J:226786 )

• altered granule neuron migration, with some granule neurons failing to migrate out of the external germinal layer, others remaining trapped in the molecular layer

• altered cerebellar granule neuron migration is already seen at 3 weeks of age

growth/size/body

decreased body size ( J:226786 )

decreased body weight ( J:226786 )

• body weight is reduced by about 40%

mortality/aging

decreased survivor rate ( J:226786 )

• mice require water gel for survival

muscle

dystonia ( J:226786 )

• mice exhibit intermittent episodes of tail extension and dystonic posturing of the body, frequently in response to handling

neoplasm

increased brain tumor incidence ( J:226786 )

• mice develop brain tumors starting at around 1 month of age

• mice that die suddenly exhibit presence of high-grade, aggressive tumors that infiltrate and obliterate the surrounding cerebellar folia

• tumors appear to arise from the cerebellum

• tumors show hallmarks of atypical teratoid/rhabdoid tumor of the central nervous system

nervous system

seizures ( J:226786 )

• mice exhibit clear seizures or intermittent electrographic seizures associated either with forelimb clonus, evolving into body jerking, and wild running or arrest of activity during the ictal EEG discharges

abnormal cerebellar granule cell migration ( J:226786 )

• altered granule neuron migration, with some granule neurons failing to migrate out of the external germinal layer, others remaining trapped in the molecular layer

• altered cerebellar granule neuron migration is already seen at 3 weeks of age

increased brain tumor incidence ( J:226786 )

• mice develop brain tumors starting at around 1 month of age

• mice that die suddenly exhibit presence of high-grade, aggressive tumors that infiltrate and obliterate the surrounding cerebellar folia

• tumors appear to arise from the cerebellum

• tumors show hallmarks of atypical teratoid/rhabdoid tumor of the central nervous system

abnormal brain white matter morphology ( J:226786 )

• defects in white matter with complete loss of tissue in regions of white matter over time

• white matter fiber tracks are reduced

• loss of white matter is already seen at 3 weeks of age

abnormal corpus callosum morphology ( J:226786 )

• defects in the corpus callosum

• lesions in the corpus callosum are already seen at 3 weeks of age

abnormal hippocampus morphology ( J:226786 )

• defects in the hippocampus

abnormal cerebral cortex morphology ( J:226786 )

• reduced cerebral cortex is already seen at 3 weeks of age

abnormal cerebellum morphology ( J:226786 )

• defects in the cytoarchitecture of the cerebellum

• progressive loss of cells and neuronal projections in the cerebellum

abnormal cerebellum external granule cell layer morphology ( J:226786 )

• aberrant external germinal layer

abnormal cerebellar layer morphology ( J:226786 )

• the boundary between the internal granule layer and the molecular layer remains diffuse

decreased oligodendrocyte number ( J:226786 )

• loss of oligodendrocytes with age

gliosis ( J:226786 )

• high level of gliosis in the fiber tracks and in the molecular layer and in the remnants of the external germinal layer

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
central nervous system cancer		DOID:3620		J:226786

Genotype
MGI:5306155

cn5

• Allelic Composition: Itgav^tm2Hyn/Itgav^tm2.1Hyn; Tg(GFAP-cre)#Gtm/0
• Genetic Background: involves: 129P2/OlaHsd * C57BL/6 * CBA

cardiovascular system

intracranial hemorrhage ( J:94376 )

• at E18 and P5, mice exhibit microhemorrhage in the cerebellum, mid-brain, and cerebral cortex unlike in control mice

cerebellum hemorrhage ( J:94376 )

• at E18 and P5, mice exhibit microhemorrhage in the cerebellum unlike in control mice

intracerebral hemorrhage ( J:94376 )

• at E18 and P5, mice exhibit microhemorrhage in the cerebral cortex unlike in control mice

• between P7 and P14, mice exhibit progressive reduction in cerebral microhemorrhage

• however, adult mice exhibit no hemorrhage or edema in the brain

nervous system

intracranial hemorrhage ( J:94376 )

• at E18 and P5, mice exhibit microhemorrhage in the cerebellum, mid-brain, and cerebral cortex unlike in control mice

cerebellum hemorrhage ( J:94376 )

• at E18 and P5, mice exhibit microhemorrhage in the cerebellum unlike in control mice

intracerebral hemorrhage ( J:94376 )

• at E18 and P5, mice exhibit microhemorrhage in the cerebral cortex unlike in control mice

• between P7 and P14, mice exhibit progressive reduction in cerebral microhemorrhage

• however, adult mice exhibit no hemorrhage or edema in the brain

Genotype
MGI:5771801

cn6

• Allelic Composition: Smarcb1^tm2Sho/Smarcb1^tm2Sho; Tg(GFAP-cre)#Gtm/0
• Genetic Background: involves: 129S1/Sv * C57BL/6 * CBA

nervous system

abnormal cerebellar granule cell migration ( J:226786 )

• variable, mild defect in granule neuron migration

abnormal brain white matter morphology ( J:226786 )

• brain lesions that are prominent in the white matter of the corpus callosum with vacuolization, spongy changes, cystic-like breakdown and destruction of tissue

• extent of damage is variable and progresses with age

abnormal corpus callosum morphology ( J:226786 )

• posterior portion of the corpus callosum is more affected than the anterior portion

thin cerebral cortex ( J:226786 )

abnormal cerebellar Purkinje cell layer ( J:226786 )

• disorganization of the Purkinje cell layer

axon degeneration ( J:226786 )

• loss of axons

demyelination ( J:226786 )

• loss of myelin

cellular

abnormal cerebellar granule cell migration ( J:226786 )

• variable, mild defect in granule neuron migration

neoplasm

neoplasm ( J:226786 )

N • mice do not develop brain tumors up to more than 1 year of age

Genotype
MGI:5292549

cn7

• Allelic Composition: Tsc1^tm1Djk/Tsc1^tm1Djk; Tg(GFAP-cre)#Gtm/0
• Genetic Background: involves: 129S4/SvJae * C57BL/6 * CBA

mortality/aging

premature death ( J:176586 )

• mice die by 6 to 10 weeks of age

nervous system

seizures ( J:176586 )

• by 6 to 10 weeks of age

behavior/neurological

seizures ( J:176586 )

• by 6 to 10 weeks of age

Genotype
MGI:5543816

cn8

• Allelic Composition: Gt(ROSA)26Sor^{tm2(tTA,CMV*1-KIAA1549/BRAF,-EGFP)Gtm}/Gt(ROSA)26Sor⁺; Tg(GFAP-cre)#Gtm/0
• Genetic Background: involves: 129S6/SvEvTac * C57BL/6 * CBA

nervous system

nervous system phenotype ( J:204469 )

N • mice exhibit normal GFAP+ and S100B+ astrocytes cell numbers

Genotype
MGI:4838320

cn9

• Allelic Composition: Nf1^tm1Par/Nf1^tm1Fcr; Tg(GFAP-cre)#Gtm/0
• Genetic Background: involves: 129S/SvEv * 129S1/Sv * 129X1/SvJ * C57BL/6 * CBA

hematopoietic system

abnormal microglial cell morphology ( J:165209 )

• at 9 months of age, mutants have significantly more microglia than controls; microglia are in the optic gliomas

immune system

abnormal microglial cell morphology ( J:165209 )

• at 9 months of age, mutants have significantly more microglia than controls; microglia are in the optic gliomas

nervous system

abnormal microglial cell morphology ( J:165209 )

• at 9 months of age, mutants have significantly more microglia than controls; microglia are in the optic gliomas

increased glioma incidence ( J:165209 )

• mice develop optic gliomas in the optic chiasm/nerves

• optic gliomas appear as gross thickenings and enlargements of the pre-chiasmic nerve at 9 months of age

abnormal astrocyte morphology ( J:165209 )

• irregularly shaped and thickened astrocytes are seen in the pre-chiasmatic optic nerves

retina ganglion cell degeneration ( J:165209 )

• retinal ganglion cell loss in the middle and peripheral region

abnormal optic nerve morphology ( J:165209 )

• optic gliomas form in the pre-chiasmatic optic nerve/optic chiasm, with increased vascularization to the area, abnormally shaped and thickened astrocytes, increase in microglia, and axonal and myelin degeneration

• mice exhibit abnormal optic nerve axon organization

abnormal optic chiasm morphology ( J:165209 )

• the optic chiasm/nerves at 9 months of age appear as gross fusiform enlargements that result in optic gliomas

• increase in vascularization in the pre-chiasmatic optic nerve and optic chiasm (and in the optic glioma)

axon degeneration ( J:165209 )

• axonal and myelin degeneration are seen in the optic nerve

abnormal astrocyte physiology ( J:165209 )

• increase in neoplastic astrocyte proliferation in the optic glioma

abnormal myelination ( J:165209 )

• disruption of the myelin is seen in the optic nerve, including degeneration and hypermyelination

hypermyelination ( J:165209 )

• hypermyelination is seen in the optic nerve

neoplasm

increased glioma incidence ( J:165209 )

• mice develop optic gliomas in the optic chiasm/nerves

• optic gliomas appear as gross thickenings and enlargements of the pre-chiasmic nerve at 9 months of age

vision/eye

retina ganglion cell degeneration ( J:165209 )

• retinal ganglion cell loss in the middle and peripheral region

abnormal optic nerve morphology ( J:165209 )

• optic gliomas form in the pre-chiasmatic optic nerve/optic chiasm, with increased vascularization to the area, abnormally shaped and thickened astrocytes, increase in microglia, and axonal and myelin degeneration

• mice exhibit abnormal optic nerve axon organization

abnormal optic chiasm morphology ( J:165209 )

• the optic chiasm/nerves at 9 months of age appear as gross fusiform enlargements that result in optic gliomas

• increase in vascularization in the pre-chiasmatic optic nerve and optic chiasm (and in the optic glioma)

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
neurofibromatosis 1		DOID:0111253	OMIM:162200	J:165209

Genotype
MGI:5292550

cn10

• Allelic Composition: Nf1^tm1Fcr/Nf1⁺; Tsc1^tm1Djk/Tsc1^tm1Djk; Tg(GFAP-cre)#Gtm/0
• Genetic Background: involves: 129S/SvEv * 129S4/SvJae * C57BL/6 * CBA

mortality/aging

premature death ( J:176586 )

• most mice die before 3 months of age

neoplasm

neoplasm ( J:176586 )

N • mice do not develop gliomas

Genotype
MGI:5292552

cn11

• Allelic Composition: Nf1^tm1Fcr/Nf1⁺; Tg(GFAP-cre)#Gtm/0
• Genetic Background: involves: 129S/SvEv * 129S4/SvJae * C57BL/6 * CBA

Nf1^tm1Fcr/Nf1⁺ Tg(GFAP-cre)#Gtm/0 mice develop prechiasmatic optic nerve and chiasmal gliomas

nervous system

abnormal neuron proliferation ( J:176586 )

• cell proliferation in the optic nerve is increased compared to in wild-type mice

increased glioma incidence ( J:176586 )

• optic gliomas

abnormal optic nerve morphology ( J:176586 )

• kinking of the prechiasmatic optic nerves

increased optic chiasm size ( J:176586 )

• enlarged

neoplasm

increased glioma incidence ( J:176586 )

• optic gliomas

vision/eye

abnormal optic nerve morphology ( J:176586 )

• kinking of the prechiasmatic optic nerves

increased optic chiasm size ( J:176586 )

• enlarged

cellular

abnormal neuron proliferation ( J:176586 )

• cell proliferation in the optic nerve is increased compared to in wild-type mice

Genotype
MGI:5292547

cn12

• Allelic Composition: Gt(ROSA)26Sor^{tm1(tTA,CMV*1-Rheb,-EGFP)Gtm}/Gt(ROSA)26Sor⁺; Tg(GFAP-cre)#Gtm/0
• Genetic Background: involves: C57BL/6 * CBA

nervous system

nervous system phenotype ( J:176586 )

N • mice exhibit normal brain weight

N • mice do not develop seizures

abnormal astrocyte physiology ( J:176586 )

• astrocytes exhibit increased mTor signaling-dependent proliferation compared to in control cells

growth/size/body

growth/size/body region phenotype ( J:176586 )

N • mice exhibit normal body weight