All Phenotypes Tg(Crx-cre)1Tfur MGI Mouse

Phenotypes associated with this allele

Jump to	Allelic Composition	Genetic Background	Genotype ID
cn1	Crb2^tm1.1Wij/Crb2^tm1.1Wij Tg(Crx-cre)1Tfur/0	involves: 129P2/OlaHsd * C57BL/6J	MGI:5586690
cn2	Spata7^tm1Mrd/Spata7^tm2Mrd Tg(Crx-cre)1Tfur/0	involves: 129S7/SvEvBrd	MGI:6386739
cn3	Atp6ap2^tm1.1Aich/Y Tg(Crx-cre)1Tfur/0	involves: C57BL/6	MGI:6357695
cn4	Gpx4^tm1Yana/Gpx4^tm1Yana Tg(Crx-cre)1Tfur/0 Tg(Gpx4)#Yana/0	involves: C57BL/6 * C57BL/6NCrlj * CBA/JNCrlj * DBA/2	MGI:6383173
cn5	Pomt1^{tm1c(EUCOMM)Hmgu}/Pomt1^{tm1c(EUCOMM)Hmgu} Tg(Crx-cre)1Tfur/0	involves: C57BL/6N	MGI:6197758
cn6	Prkci^tm1Kido/Prkci^tm1Kido Tg(Crx-cre)1Tfur/0	Not Specified	MGI:3609222

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

nervous system

hydrocephaly ( J:210675 )

• 42% incidence of severe hydrocephalus

dilated brain ventricle ( J:210675 )

• ventricles of newborns with hydrocephalus are dilated and the subventricular structures are severely damaged

abnormal Muller cell morphology ( J:210675 )

• disruption of the apical end-feet of Muller glial cells at 3 months of age

short retina rod cell outer segment ( J:210675 )

• at 3 months of age, some areas show rod photoreceptors with short or absent outer segments and rhodopsin accumulation in the cell soma

• rod bipolar cells are misplaced in the outer nuclear layer in areas with photoreceptor protrusions at 3 months of age

abnormal retina cone cell outer segment morphology ( J:210675 )

• at 3 months of age, the cones show absence of or shortened outer segments and some of the nuclei are displaced to a basal part of the outer nuclear layer, close to the outer plexiform layer

short retina cone cell outer segment ( J:210675 )

vision/eye

abnormal ocular fundus morphology ( J:210675 )

• several spots and patchy areas are seen in fundus imaging of 1 month old mice

abnormal retina morphology ( J:210675 )

• lamination of the retina at P10 is severely affected and reduction of the thickness of the outer retina is seen at 1 month of age

• distribution and localization of adherens junctions in the retina are irregular at P3

retina neovascularization ( J:210675 )

• retinal vasculature is abnormal at 1 month of age, with mice showing sites of neovascularization

abnormal Muller cell morphology ( J:210675 )

• disruption of the apical end-feet of Muller glial cells at 3 months of age

abnormal retina layer morphology ( J:210675 )

• protrusion of photoreceptor cells into the subretinal space is seen as early as P1, P3 and P10 and misplaced inner retinal cells in the neuroepithelial layer and adjacent to the retinal pigment epithelium are seen at E18.5

abnormal retina pigment epithelium morphology ( J:210675 )

• misplaced inner retinal cells in the neuroepithelial layer and adjacent to the retinal pigment epithelium are seen at E18.5

• microvilli from the retinal epithelium cells are compressed between the ectopic photoreceptor nuclei and the apical membrane of the retinal pigment epithelium at P3

abnormal retina ganglion layer morphology ( J:210675 )

• at P10, ectopic nuclei are seen in the ganglion cell layer

abnormal retina outer nuclear layer morphology ( J:210675 )

• at 1 and 3 months of age, some residual large photoreceptor rosettes and retinal folds are seen in the outer nuclear layer

• ectopic Sox9-positive Muller glial cell nuclei are seen it the top of the outer nuclear layer

thin retina outer nuclear layer ( J:210675 )

• the outer nuclear layer is reduced to 4-6 rows of photoreceptor nuclei at 3 months of age

abnormal retina outer plexiform layer morphology ( J:210675 )

• the outer plexiform layer is fragmented at 3 months of age

• misplaced inner retinal cells in the outer plexiform layer at 1 month of age

abnormal retina photoreceptor layer morphology ( J:210675 )

• at P3, rosettes composed of photoreceptor cells in the middle of the neuroepithelial layer and rosettes composed of inner retinal cells at the top of the neuroepithelial layer are seen in the periphery of the retina

• photoreceptor rosettes are occasionally seen in the neuroepithelial layer at the periphery of the retina as early as E15.5

short retina rod cell outer segment ( J:210675 )

• at 3 months of age, some areas show rod photoreceptors with short or absent outer segments and rhodopsin accumulation in the cell soma

• rod bipolar cells are misplaced in the outer nuclear layer in areas with photoreceptor protrusions at 3 months of age

abnormal retina cone cell outer segment morphology ( J:210675 )

• at 3 months of age, the cones show absence of or shortened outer segments and some of the nuclei are displaced to a basal part of the outer nuclear layer, close to the outer plexiform layer

short retina cone cell outer segment ( J:210675 )

abnormal retina outer limiting membrane morphology ( J:210675 )

• disruptions of the outer limiting membrane are seen at the periphery as early as E18.5 and P3

increased susceptibility to age-related retinal degeneration ( J:210675 )

• progressive retinal degeneration is seen with increasing age, characterized by an increase in the number of fundus abnormalities and a more pronounced thinning of the retina

• at 5 months of age, some areas of the retina exhibit regions devoid of photoreceptors

retina fold ( J:210675 )

• at 1 and 3 months of age, some retinal folds are seen in the outer nuclear layer

abnormal electroretinogram waveform feature ( J:210675 )

• by 1 month of age, retinas show a reduction in both scotopic and photopic ERG amplitudes, indicating altered rod and cone physiology

decreased a-wave amplitude ( J:210675 )

• at high stimulus intensities under scotopic conditions, the a-wave is more reduced than the b-wave, resulting in a high b/a ratio and indicating a primary defect in photoreceptors

cardiovascular system

retina neovascularization ( J:210675 )

• retinal vasculature is abnormal at 1 month of age, with mice showing sites of neovascularization

pigmentation

abnormal retina pigment epithelium morphology ( J:210675 )

• misplaced inner retinal cells in the neuroepithelial layer and adjacent to the retinal pigment epithelium are seen at E18.5

• microvilli from the retinal epithelium cells are compressed between the ectopic photoreceptor nuclei and the apical membrane of the retinal pigment epithelium at P3

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
retinitis pigmentosa		DOID:10584	OMIM:PS268000	J:210675

Allelic Composition	Spata7^tm1Mrd/Spata7^tm2Mrd Tg(Crx-cre)1Tfur/0
Genetic Background	involves: 129S7/SvEvBrd

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Spata7^tm1Mrd mutation (1 available); any Spata7 mutation (27 available)
Spata7^tm2Mrd mutation (0 available); any Spata7 mutation (27 available)
Tg(Crx-cre)1Tfur mutation (1 available)

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

vision/eye

retina photoreceptor degeneration ( J:279827 )

• progressive degeneration that is significant at P28 that is not as pronounced as in Spata7^tm2Mrd homozygotes

thin retina outer nuclear layer ( J:279827 )

• 30% reduction at P28

• 8 rows at 4 months compared with 13 in control mice

• 4 rows at 7 months compared with 13 in control mice

abnormal cone electrophysiology ( J:279827 )

• reduced b-wave at P28 and 4 months

abnormal rod electrophysiology ( J:279827 )

• reduced a-wave at P28

• reduced a-wave and b-wave at 4 months

nervous system

retina photoreceptor degeneration ( J:279827 )

• progressive degeneration that is significant at P28 that is not as pronounced as in Spata7^tm2Mrd homozygotes

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
Leber congenital amaurosis 3		DOID:0110331	OMIM:604232	J:279827

Allelic Composition	Atp6ap2^tm1.1Aich/Y Tg(Crx-cre)1Tfur/0
Genetic Background	involves: C57BL/6

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Atp6ap2^tm1.1Aich mutation (0 available); any Atp6ap2 mutation (10 available)
Tg(Crx-cre)1Tfur mutation (1 available)

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

vision/eye

abnormal retina morphology ( J:204145 )

• cellular defects are seen in the retina at E14.5

• marker analysis indicates that retinal progenitor distribution is disturbed in photoreceptor cells and cell adhesion and polarity molecules are mislocalized in photoreceptors, however cell fate determination of photoreceptors is not affected

abnormal retina layer morphology ( J:204145 )

• retinal lamination is disrupted with plexiform patches and sporadic rosette-like structures in the outer nuclear layer at P14

retina photoreceptor degeneration ( J:204145 )

• loss of photoreceptor cells

decreased total retina thickness ( J:204145 )

• retinal thinning is seen at E14.5 but not at E12.5

decreased a-wave amplitude ( J:204145 )

• dark-adapted ERG responses show reduced a-wave amplitudes

decreased b-wave amplitude ( J:204145 )

• dark-adapted ERG responses show reduced b-wave amplitudes

nervous system

retina photoreceptor degeneration ( J:204145 )

• loss of photoreceptor cells

Allelic Composition	Gpx4^tm1Yana/Gpx4^tm1Yana Tg(Crx-cre)1Tfur/0 Tg(Gpx4)#Yana/0
Genetic Background	involves: C57BL/6 * C57BL/6NCrlj * CBA/JNCrlj * DBA/2

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Gpx4^tm1Yana mutation (0 available); any Gpx4 mutation (82 available)
Tg(Crx-cre)1Tfur mutation (1 available)
Tg(Gpx4)#Yana mutation (0 available)

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

vision/eye

abnormal retina morphology ( J:182776 )

• lipid peroxidation is increased in inner segment photoreceptor cells at P12, and a modest increase is seen in the other layers of the retina including the inner nuclear layer and ganglion cell layer

abnormal photoreceptor connecting cilium morphology ( J:182776 )

• 80% decrease in the number of connecting cilia in photoreceptor cells

abnormal photoreceptor inner segment morphology ( J:182776 )

• mitochondrial area in inner segment of photoreceptor cells is decreased indicating a decrease in mitochondrial biomass at P11

abnormal photoreceptor outer segment morphology ( J:182776 )

• the outer segment layer is very thin

disorganized photoreceptor outer segment ( J:182776 )

• the outer segment is disorganized and consists of cell membranes that branch and form a large web-like network structure without apparent disc rim and an abnormally wide intra- and inter-outer segment membrane space

thin retina inner nuclear layer ( J:182776 )

• milder thinning or degeneration of the inner nuclear layer

retina outer nuclear layer degeneration ( J:182776 )

• rapid loss of the outer nuclear layer

• an increasing number of TUNEL+ cells are seen in the outer nuclear layer after P12

retina degeneration ( J:182776 )

• mice show rapid and massive retinal degeneration after P12 with none of the photoreceptor cells retained by P21

• cone photoreceptor cells are lost earlier than rod photoreceptor cells

nervous system

abnormal photoreceptor connecting cilium morphology ( J:182776 )

• 80% decrease in the number of connecting cilia in photoreceptor cells

abnormal photoreceptor inner segment morphology ( J:182776 )

• mitochondrial area in inner segment of photoreceptor cells is decreased indicating a decrease in mitochondrial biomass at P11

abnormal photoreceptor outer segment morphology ( J:182776 )

• the outer segment layer is very thin

disorganized photoreceptor outer segment ( J:182776 )

cellular

abnormal photoreceptor connecting cilium morphology ( J:182776 )

• 80% decrease in the number of connecting cilia in photoreceptor cells

decreased mitochondrial DNA content ( J:182776 )

• mitochondrial DNA replication in the whole retina is lower

Allelic Composition	Pomt1^{tm1c(EUCOMM)Hmgu}/Pomt1^{tm1c(EUCOMM)Hmgu} Tg(Crx-cre)1Tfur/0
Genetic Background	involves: C57BL/6N
Cell Lines	HEPD0522_6_C03, HEPD0522_6_F02

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Pomt1^{tm1c(EUCOMM)Hmgu} mutation (1 available); any Pomt1 mutation (48 available)
Tg(Crx-cre)1Tfur mutation (1 available)

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

vision/eye

abnormal retina bipolar cell morphology ( J:263043 )

• abnormal sprouting of dendrites from bipolar cells protruding into the outer nuclear layer

thin retina outer nuclear layer ( J:263043 )

• thickness of the outer nuclear layer is reduced

abnormal retina outer plexiform layer morphology ( J:263043 )

• loss of the normal arrangement of synaptic proteins in the outer half of the outer plexiform layer

thin retina outer plexiform layer ( J:263043 )

• thickness of the outer plexiform layer is reduced

increased b-wave implicit time ( J:263043 )

• significant delay in the scotopic and mesopic b-waves, as reflected by their longer implicit time

• increase in the photopic b-wave implicit time

decreased b-wave amplitude ( J:263043 )

• under scotopic conditions, the ERG b-wave elicited in dark-adopted mice is severely reduced, with decreased b-wave amplitude

• under photopic conditions, the ERG b-wave elicited in light-adapted mice is lower than in controls for any tested light intensity

• however, a-waves are similar to control mice, indicating normal function of photoreceptors

abnormal vision ( J:263043 )

• in the water maze, mice fail to learn and do not improve their success rate due to visual impairment

behavior/neurological

abnormal optokinetic reflex ( J:263043 )

• responses induced by screen rotation at different spatial frequencies of alternating white and black stripes are lower and contrast sensitivity in the range 0.044-0.177 cycles/degree is weaker than in controls, indicating a strong visual impairment

nervous system

abnormal innervation ( J:263043 )

• dendritic projections from bipolar and horizontal cells are altered

• horizontal cell dendritic projections barely reach their location laterally to the synaptic ribbon and bipolar cell dendritic projections fail to meet photoreceptor synaptic terminals

abnormal axon morphology ( J:263043 )

• shortening of cone axons

abnormal synaptic bouton morphology ( J:263043 )

• misalignment of presynaptic terminals of photoreceptors in the outer plexiform layer in specific areas of the retina

• mitochondria in both rod and cone axon terminals are reduced in number, appear swollen and show severe disruption of cristae

abnormal dendrite morphology ( J:263043 )

• abnormal sprouting of dendrites from bipolar cells protruding into the outer nuclear layer

abnormal retina bipolar cell morphology ( J:263043 )

• abnormal sprouting of dendrites from bipolar cells protruding into the outer nuclear layer

abnormal ribbon synapse morphology ( J:263043 )

• dendritic processes of bipolar and horizontal cells are not inserted into the invaginations of ribbon synapses

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
muscular dystrophy-dystroglycanopathy type B1		DOID:0050588	OMIM:613155	J:263043

Allelic Composition	Prkci^tm1Kido/Prkci^tm1Kido Tg(Crx-cre)1Tfur/0
Genetic Background	Not Specified

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Prkci^tm1Kido mutation (0 available); any Prkci mutation (69 available)
Tg(Crx-cre)1Tfur mutation (1 available)

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

vision/eye

microphthalmia ( J:102364 )

• mild microphthalmia

abnormal retina morphology ( J:102364 )

• severe laminar disorganization of the entire retina with all types of cells, including mitotic progenitors and S-phase progenitors, randomly distributed

• apical junctions are not observed at the retinal apical edge nor around scattered photoreceptors indicating that adherens junction formation between the immature photoreceptors and the progenitors is lost

abnormal retina photoreceptor morphology ( J:102364 )

• photoreceptors are randomly distributed in the retina, indicating loss of photoreceptor polarity

• outer segment, inner segment, or synaptic terminal structures are absent although components of the outer segment, such as rhodopsin and S-opsin, and synaptic ribbons are detected

absent photoreceptor inner segment ( J:102364 )

• absent although components such as are rhodopsin and S-opsin are detected

absent photoreceptor outer segment ( J:102364 )

• absent outer segment

nervous system

hydrocephaly ( J:102364 )

abnormal retina photoreceptor morphology ( J:102364 )

• photoreceptors are randomly distributed in the retina, indicating loss of photoreceptor polarity

• outer segment, inner segment, or synaptic terminal structures are absent although components of the outer segment, such as rhodopsin and S-opsin, and synaptic ribbons are detected

absent photoreceptor inner segment ( J:102364 )

• absent although components such as are rhodopsin and S-opsin are detected

absent photoreceptor outer segment ( J:102364 )

• absent outer segment

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)
Citing These Resources Funding Information Warranty Disclaimer, Privacy Notice, Licensing, & Copyright Send questions and comments to User Support.	last database update 11/12/2024 MGI 6.24