Analysis Tools|
Allele Symbol Allele Name Allele ID |
Tg(Chx10-EGFP/cre,-ALPP)2Clc transgene insertion 2, Constance L Cepko MGI:3052237 |
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| Summary |
26 genotypes
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| ♀ | phenotype observed in females |
| ♂ | phenotype observed in males |
| N | normal phenotype |
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• starburst amacrine cells (SACs) have defects in differentiation as determined by decrease expression of Chat and Slc18a3
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• starburst amacrine cells (SACs) have defects in differentiation as determined by decrease expression of Chat and Slc18a3
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| ♀ | phenotype observed in females |
| ♂ | phenotype observed in males |
| N | normal phenotype |
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• mice show progressive alteration and remodeling of the laminar retinal structure from P16 to P45
• however, no major defects in neuroepithelial cell polarity or in structure of adherens junctions are seen in immature retinas at P2
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• mice show the formation of photoreceptor rosettes at P16 (circular structures comprising photoreceptors only that are oriented toward an internal lumen with photoreceptor outer segments protruding inward) that increase in number from P16 and P45 and decrease in size and eventually disappear by 3 months of age when degeneration of photoreceptors begins
• rosettes are scattered along the retinal outer surface and are composed of photoreceptors and their synaptic terminals
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• photoreceptors with rosettes degenerate as mice reach 3 months of age
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• the outer nuclear layer becomes progressively thinner; it is thinner in the peripheral compared with the central retina, indicating a periphery-to-central degenerative gradient
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• formation of photoreceptor rosettes at P16 progresses to more general cellular disorganization
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• widespread degeneration of retinal cell types with age
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• Muller cells become hypertrophic with radial processes increasing in size and GFAP reactivity increasing at later stages during when loss of retinal cells is occurring, indicating glial activation
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• scotopic a wave amplitude is diminished at 1, 3, and 5 months of age
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• scotopic and photopic b wave amplitudes are diminished
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• mice show the formation of photoreceptor rosettes at P16 (circular structures comprising photoreceptors only that are oriented toward an internal lumen with photoreceptor outer segments protruding inward) that increase in number from P16 and P45 and decrease in size and eventually disappear by 3 months of age when degeneration of photoreceptors begins
• rosettes are scattered along the retinal outer surface and are composed of photoreceptors and their synaptic terminals
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• photoreceptors with rosettes degenerate as mice reach 3 months of age
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| ♀ | phenotype observed in females |
| ♂ | phenotype observed in males |
| N | normal phenotype |
| N |
• no morphological abnormalities are seen in the retina
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• scotopic a wave amplitude is reduced at 1, 3, and 5 months of age
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• scotopic and photopic b wave amplitudes are reduced at 1, 3, and 5 months of age
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| ♀ | phenotype observed in females |
| ♂ | phenotype observed in males |
| N | normal phenotype |
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• retinoblastoma cells that express amacrine/horizontal cell markers also extend processes and form synapses; some of these Golgi-Cox-labeled cells extend 1-3 long main processes with further neurite branching characteristic of horizontal or wide-field amacrine cells, while more (nearly half of ) labeled cells extend a main process with extensive neurite outgrowth characteristic of amacrine cells, and the remaining cells are less differentiated with short, unbranched neurites
• these labeled cells are mainly found near the tumor origin, while fewer are present toward the lens and anterior chamber
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• tumor cells that invade the anterior eye chamber beneath the cornea are densely packed stage II cells surrounded by sparse regions of plexus with no synaptic densities or vesiclesin in the plexus or rosettes of these cells
• tumor cells have abundant mitochondria and mitotic figures; some rosettes have a central plexus made up of large, undifferentiated processes, with other rosettes having a central plexus containing neurons and synapses
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• areas of the plexus within the posterior chamber are composed of neuron-like processes having synaptic structures similar to horizontal/amacrine cells; this is seen in extensive plexus areas of tumors
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• retinoblastoma cells that express amacrine/horizontal cell markers also extend processes and form synapses; some of these Golgi-Cox-labeled cells extend 1-3 long main processes with further neurite branching characteristic of horizontal or wide-field amacrine cells, while more (nearly half of ) labeled cells extend a main process with extensive neurite outgrowth characteristic of amacrine cells, and the remaining cells are less differentiated with short, unbranched neurites
• these labeled cells are mainly found near the tumor origin, while fewer are present toward the lens and anterior chamber
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• a substantial proportion of tumor cells expressing amacrine/horizontal cell markers are proliferating as shown by labeled thymidine incorporation
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| ♀ | phenotype observed in females |
| ♂ | phenotype observed in males |
| N | normal phenotype |
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• tumor cells that invade the anterior eye chamber beneath the cornea are densely packed stage II cells surrounded by sparse regions of plexus with no synaptic densities or vesiclesin in the plexus or rosettes of these cells
• tumor cells have abundant mitochondria and mitotic figures; some rosettes have a central plexus made up of large, undifferentiated processes, with other rosettes having a central plexus containing neurons and synapses
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• areas of the plexus within the posterior chamber are composed of neuron-like processes having synaptic structures similar to horizontal/amacrine cells; this is seen in smaller areas of plexus in tumors
• processes are usually smaller in diameter (<0.5 um) but large ones of 1-3 um are observed occasionally; variety of synaptic arrangements occur and all types can be found in contacts among processes, while ribbon synapses are seen only in areas near photoreceptor cell bodies
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• significant disruptions in retinal morphology are observed by P12 to 13
• in diseased eyes, retina blastoma cells rupture the inner limiting membrane (ILM), particularly by the apex of vitreal protrusions that may be present; at these points, tumor cell bodies and associated vasculature can be seen
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• mice have much more aggressive, invasive form of retinoblastoma than Trp53-sufficient compound mutants; tumor size varies with age and genotype
• an early-stage tumor displayed a pronounced vitreal protrusion in one animal, and contained structures resembling neuronal processes like those in the plexiform layer of the normal retina
• in tumors, proteins usually found in amacrine/horizontal cells, including Gad65, Snap25, Calbindin, vGlut-1, E-cadherin, N-cadherin, synapsin, and Syntaxin-1 are expressed; these cells are considered to be differentiated
• cells positive for amacrine/horizontal cell markers (1.7-3.6 x 106 cells/tumor) are much more numerous than number of amacrine/horizontal cells in a normal retina
• regions of tumors negative for amacrine/horizontal markers contain more densely packed nuclei
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• mice have much more aggressive, invasive form of retinoblastoma than Trp53-sufficient compound mutants; tumor size varies with age and genotype
• an early-stage tumor displayed a pronounced vitreal protrusion in one animal, and contained structures resembling neuronal processes like those in the plexiform layer of the normal retina
• in tumors, proteins usually found in amacrine/horizontal cells, including Gad65, Snap25, Calbindin, vGlut-1, E-cadherin, N-cadherin, synapsin, and Syntaxin-1 are expressed; these cells are considered to be differentiated
• cells positive for amacrine/horizontal cell markers (1.7-3.6 x 106 cells/tumor) are much more numerous than number of amacrine/horizontal cells in a normal retina
• regions of tumors negative for amacrine/horizontal markers contain more densely packed nuclei
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| ♀ | phenotype observed in females |
| ♂ | phenotype observed in males |
| N | normal phenotype |
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• mice with retinoblastoma tumors are characterized by pronounced loss of photoreceptor cells
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• retinoblastoma cells that express amacrine/horizontal cell markers also extend processes and form synapses; some of these Golgi-Cox-labeled cells extend 1-3 long main processes with further neurite branching characteristic of horizontal or wide-field amacrine cells, while more (nearly half of ) labeled cells extend a main process with extensive neurite outgrowth characteristic of amacrine cells, and the remaining cells are less differentiated with short, unbranched neurites
• these labeled cells are mainly found near the tumor origin, while fewer are present toward the lens and anterior chamber
• tumors show appearance of unique populations of undifferentiated tumor-like cells, and extensive formation of plexiform regions
• in a large tumor that filled much of the vitreal space contained two cell types: some cells are stage I retinoblastoma cells with pale, round nuclei resembling differentiated neurons and always associated with a plexus or tightly-packed stage II retinoblastoma cells with irregular nuclei with little or no plexus associated with individual cells
• rosettes in retinoblastomas are usually composed of stage I cells with a central plexus, but are adjacent to clusters of stage II cells
• plexus regions of tumors show mitotic figures and apoptotic cells; plexus regions also contained synaptic densities and associated synaptic vesicles
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• areas of the plexus within the posterior chamber are composed of neuron-like processes having synaptic structures similar to horizontal/amacrine cells; this is seen in smaller areas of plexus in tumors
• processes are usually smaller in diameter (<0.5 um) but large ones of 1-3 um are observed occasionally; variety of synaptic arrangements occur and all types can be found in contacts among processes, while ribbon synapses are seen only in areas near photoreceptor cell bodies
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• significant disruptions in retinal morphology are observed by P12 to 13
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• retinoblastoma cells that express amacrine/horizontal cell markers also extend processes and form synapses; some of these Golgi-Cox-labeled cells extend 1-3 long main processes with further neurite branching characteristic of horizontal or wide-field amacrine cells, while more (nearly half of ) labeled cells extend a main process with extensive neurite outgrowth characteristic of amacrine cells, and the remaining cells are less differentiated with short, unbranched neurites
• these labeled cells are mainly found near the tumor origin, while fewer are present toward the lens and anterior chamber
• tumors show appearance of unique populations of undifferentiated tumor-like cells, and extensive formation of plexiform regions
• in a large tumor that filled much of the vitreal space contained two cell types: some cells are stage I retinoblastoma cells with pale, round nuclei resembling differentiated neurons and always associated with a plexus or tightly-packed stage II retinoblastoma cells with irregular nuclei with little or no plexus associated with individual cells
• rosettes in retinoblastomas are usually composed of stage I cells with a central plexus, but are adjacent to clusters of stage II cells
• plexus regions of tumors show mitotic figures and apoptotic cells; plexus regions also contained synaptic densities and associated synaptic vesicles
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• mice with retinoblastoma tumors are characterized by pronounced loss of photoreceptor cells
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| ♀ | phenotype observed in females |
| ♂ | phenotype observed in males |
| N | normal phenotype |
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• mice have much more aggressive, invasive form of retinoblastoma than Trp53-sufficient compound mutants, but onset is delayed compared to that observed in Rbl1 homozygous compound mutants
• tumor size varies with age and genotype
• mice show rapid filling of the vitreal cavity with densely packed tumor cells and rosettes; in the tumors, there is an overabundance of stage II retinoblastoma cells
• near outer surface of retina near tumor origin site, there are regions of plexus with synaptic densities and synaptic vesicles, indistinguishable from the Rb1;Rbl1 mutants that are wild-type for Trp53
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• significant disruptions in retinal morphology are observed by P12 to 13
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• significant disruptions in retinal morphology are observed by P12 to 13
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| ♀ | phenotype observed in females |
| ♂ | phenotype observed in males |
| N | normal phenotype |
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• morphological alterations after E18.5
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• numerous large retinal blood vessels by 12 months of age
• sites of neovascularization by 3 months of age, sometimes with choroidal structures
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• misplaced bipolar cell nuclei in the outer nuclear layer
• cells possess fewer dendrites than in controls
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• disruption of the retinal pigment layer by 3 months
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• reduced retinal thickness in the outer retina and cellular mislocalization
• most severe degeneration in the central retina
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• normal orientation of photoreceptors is lost
• rosettes and half rosettes found in the periphery of the retina
• ectopic nuclei in subretinal space at 10 days of age, do not develop proper segments
• loss of adherens junctions near ectopic cells
• increased apoptosis between 3 and 21 days
• apoptosis level peaks around 15 days
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• very few photoreceptor cells by 12 months
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• cone cells also displaced to sub retinal space
• polarity defects
• cone outer segments shorter than in controls
• cells survive to 3 months but with loss of segments
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• sporadic disruptions of the outer limiting membrane
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• accumulation of autofluorescent remains of lost photoreceptor cells
• indications of progressive retinal degeneration by 1 month of age
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• progressive signal amplitude reduction with age to near extinction at 18 months of age
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• reduced amplitude of photopic electroretinography responses
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• reduced amplitude of scotopic electroretinography responses
• greater attenuation of a-waves relative to b-waves at high stimulus intensities (higher b/a ratio)
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• very few photoreceptor cells by 12 months
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• misplaced bipolar cell nuclei in the outer nuclear layer
• cells possess fewer dendrites than in controls
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• cone cells also displaced to sub retinal space
• polarity defects
• cone outer segments shorter than in controls
• cells survive to 3 months but with loss of segments
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• disruption of the retinal pigment layer by 3 months
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• numerous large retinal blood vessels by 12 months of age
• sites of neovascularization by 3 months of age, sometimes with choroidal structures
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Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
| retinitis pigmentosa | DOID:10584 |
OMIM:PS268000 |
J:191149 | |
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| ♀ | phenotype observed in females |
| ♂ | phenotype observed in males |
| N | normal phenotype |
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| ♀ | phenotype observed in females |
| ♂ | phenotype observed in males |
| N | normal phenotype |
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• phenotype similar to that when Tg(rx3-icre)1Mjam was used for cre expression
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• sporadic loss of photoreceptors at 18 months
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• areas of disruption and ectopic photoreceptors at 18 months
• slight thinning of layer at 6, 12, 18 months
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• phenotype similar to that when Tg(rx3-icre)1Mjam was used for cre expression
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| ♀ | phenotype observed in females |
| ♂ | phenotype observed in males |
| N | normal phenotype |
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• four fold increase in degenerative spots on retinas compared to controls
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| ♀ | phenotype observed in females |
| ♂ | phenotype observed in males |
| N | normal phenotype |
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• at P5, there is a decrease in the number of apoptotic cells in the retina
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• the number of apoptotic cells is increased in the retina at E17.5
• however, at P5, there is a decrease in the number of apoptotic cells
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• many spots and patchy areas are visible throughout the retina (pseudo-rosettes)
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• disruption of the apical adherens junctions/subapical region in retinas at E15.5 which leads to ectopic localization of some photoreceptor and bipolar cells in the ganglion cell layer and ganglion, amacrine, and bipolar cells in the outer nuclear layer
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• ganglion cell nuclei and inner nuclear layer cells are seen in the outer nuclear layer and some photoreceptor nuclei are seen in the ganglion cell layer
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• rods, cones and bipolar cells localize ectopically in the ganglion cell layer
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• amacrine and ganglion cells surrounded by bipolar cells form pseudo-rosettes in the photoreceptor layer
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• the outer limiting membrane is perturbed at the periphery of the retina at E15.5, and progressively extends to the center of the retinas where rosettes form
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• the number of mitotic cells is increased in the retina at E17.5 and at P5
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• at P5, there is a decrease in the number of apoptotic cells in the retina
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• the number of apoptotic cells is increased in the retina at E17.5
• however, at P5, there is a decrease in the number of apoptotic cells
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• mice exhibit a greater reduction in amplitudes of electroretinogram responses at 1 month of age than single Crb2 conditional mutants, with both scotopic and photopic responses
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Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
| Leber congenital amaurosis 8 | DOID:0110079 |
OMIM:613835 |
J:207895 | |
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| ♀ | phenotype observed in females |
| ♂ | phenotype observed in males |
| N | normal phenotype |
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• between E15.5 and E17.5, the adherens junctions are gradually lost in the neural retina
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• increase in the number of Sox9-positive Muller cells
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• increase in the number of GABAergic amacrine cells and late born GlyT1 positive amacrine cells
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• increase in the number of Chx10-positive bipolar cells
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• increase in population of late progenitor cells and late born cells due to dysregulation of the cell cycle at E17.5
• between E15.5 and E17.5, the nuclei of the retinal progenitors show abnormal orientation
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• aberrant layering in the retina is seen at 1 month of age, with a single inner plexiform layer, an abnormal thick ganglion cell layer and a second broad nuclear layer
• all retinal cell types are generated but a separate photoreceptor nuclear layer, inner and outer segment layer and outer plexiform layer are not formed
• cellular mislocalization of late born cells (rod photoreceptors, Muller cells, and bipolar cells) near the retinal pigment epithelium are seen at E13.5
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• no distinct photoreceptor layer is formed at P5
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• increase in the number of rods at P10
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• severe disorganization of the retina
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• the outer limiting membrane is perturbed at E13.5
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• retinal vasculature defects leading to the thinning of the retinas at 3-6 months of age
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• increase in retina thickness at P10 and P14 due to excessive proliferation of late-born retinal progenitor cells
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• retinas degenerate rapidly after 1 month
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• from E15.5 onwards, the number of M-phase cells is increased in retinas
• proportion of retinal cells in G1 is reduced and proportion of cells in S and G2/M is increased at E17.5, however at P1 and P5, cells return to normal cell cycle
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• increase in the number of apoptotic cells in the retinas at P10 and P14 (rod photoreceptors), and 3 months (bipolar cells)
• the number of apoptotic cells in the retina is increased at E13.5 and E17.5 onwards
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• mice exhibit a greater reduction in amplitudes of electroretinogram responses at 1 month of age than single Crb2 conditional mutants, with both scotopic and photopic responses
• electroretinogram responses are below detection at 3 and 6 months of age
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• increase in the number of apoptotic cells in the retinas at P10 and P14 (rod photoreceptors), and 3 months (bipolar cells)
• the number of apoptotic cells in the retina is increased at E13.5 and E17.5 onwards
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• increase in the number of Sox9-positive Muller cells
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• increase in the number of GABAergic amacrine cells and late born GlyT1 positive amacrine cells
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• increase in the number of rods at P10
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• increase in the number of Chx10-positive bipolar cells
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Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
| Leber congenital amaurosis 8 | DOID:0110079 |
OMIM:613835 |
J:207895 | |
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| ♀ | phenotype observed in females |
| ♂ | phenotype observed in males |
| N | normal phenotype |
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• mice exhibit a milder phenotype than in Tg(RNU6-RNAi:Mpp5)13Wij Tg(rx3-icre)1Mjam mice
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• at 3 months of age, mice exhibit ectopic photoreceptor nuclei in the subretinal space unlike wild-type mice
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• at 12 months, the outer plexiform layer exhibit lamination defects
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• thinner at 12 months of age
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• in the outer plexiform layer
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• with sporadic gaps at 3 months of age
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• mice exhibit slightly reduced photopic response compared with wild-type mice
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• mice exhibit slightly reduced scotopic response compared with wild-type mice
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• in the outer plexiform layer
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| ♀ | phenotype observed in females |
| ♂ | phenotype observed in males |
| N | normal phenotype |
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• recombined but not non-recombined dopaminergic amacrine cells and neurites are clumped
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• recombined but not non-recombined dopaminergic amacrine cells and neurites are clumped
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• recombined but not non-recombined dopaminergic amacrine cells and neurites are clumped
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• recombined but not non-recombined dopaminergic amacrine cells and neurites are clumped
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| ♀ | phenotype observed in females |
| ♂ | phenotype observed in males |
| N | normal phenotype |
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• under dark-adapted conditions, amplitude of b-wave is reduced by 27%, causing significant reduction in b-wave to a-wave amplitude ratio
• under light-adapted conditions, b-wave amplitude is reduced by 44%
• no detectable circadian rhythm of b-wave amplitude is observed, with b-wave responses apparently fixed at low amplitude at times of both high and low b-wave amplitude (circadian times CT 6 and CT 18)
• no circadian rhythm of b-wave implicit time (time between light stimulus and peak of b-wave) is detectable, whereas this is robust in controls
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| ♀ | phenotype observed in females |
| ♂ | phenotype observed in males |
| N | normal phenotype |
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• at E12.5, 13.5, and 18.0, cell death is significantly elevated; increased apoptosis is observed primarily in central retina at E12.5 and 13.5 while periphery is affected at E18.0
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• thin optic nerve
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• newborn mice have small eyes
• total weight of eyes is 50% of wild-type
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• size of retina is decreased compared to wild-type
• retina is detached from retinal pigment epithelium (RPE), while abutting the lens
• eyes analyzed at P7 following BrdU injection at E16 show reduced cell numbers in outer nuclear layer (ONL), inner nuclear layer (INL), and ganglion cell layer (GCL)
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• by P7, cell number is significantly reduced
• 2-fold decrease in BrdU-labeled cells is observed at P7 (BrdU injection at E16)
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• by P7, cell number is significantly reduced
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• inner limiting membrane (ILM) structure is perturbed; retina has dysmorphic fibrous layer instead of well-organized ILM
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• ganglion cell layer (GCL) exhibits decreased cellularity in postnatal mice
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• by P7, few ganglion cells are detected in retina
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• by P14, rosettes are observed in retina
• ciliary body is fused to retina, so that it could not be separated from neural retina
• almost complete retinal degeneration has occurred by P30; entire eye is absent in mutants
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• by P7, cell number is significantly reduced
• 2-fold decrease in BrdU-labeled cells is observed at P7 (BrdU injection at E16)
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• by P7, few ganglion cells are detected in retina
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• by P7, cell number is significantly reduced
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• thin optic nerve
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• at E12.5, 13.5, and 18.0, cell death is significantly elevated; increased apoptosis is observed primarily in central retina at E12.5 and 13.5 while periphery is affected at E18.0
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| ♀ | phenotype observed in females |
| ♂ | phenotype observed in males |
| N | normal phenotype |
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• about a 90% reduction in dopamine levels in the retina
• L-DOPA treatment rescues the visual defects
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• overall light-adapted b-wave amplitudes are reduced and the amplitude of the day/night rhythm is damped compared to controls in a light-adapted electroretinographic assay
• on day 2 in constant darkness, circadian regulation of the light-adapted electroretinographic response is abolished
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• optokinetic tracking indicates a decrease in contrast sensitivity
• visual acuity is reduced
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• about a 90% reduction in the retina
• however, dopamine levels in the brain are preserved
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| ♀ | phenotype observed in females |
| ♂ | phenotype observed in males |
| N | normal phenotype |
| N |
• mice exhibit normal inner nuclear, inner plexiform and ganglion cell layer thickness and numbers of bipolar cells
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| ♀ | phenotype observed in females |
| ♂ | phenotype observed in males |
| N | normal phenotype |
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• 40% thinner than in wild-type mice
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• 40% thinner than in wild-type mice
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• the retina is 25% thinner than in wild-type mice
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| ♀ | phenotype observed in females |
| ♂ | phenotype observed in males |
| N | normal phenotype |
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• retinal apoptosis during the first two weeks after birth is increased compared to in wild-type mice
• increased apoptosis is observed in the neuroblast and ganglion cell layers at P0 and is confined to the inner nuclear layer at P7
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• by P14, 40% thinner than in wild-type mice
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• by P14, 40% thinner than in wild-type mice
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• retinal ganglion cells fire at a lower rate in response to flicker stimuli and the time course of the light response is lower than in wild-type mice
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• retinal apoptosis during the first two weeks after birth is increased compared to in wild-type mice
• increased apoptosis is observed in the neuroblast and ganglion cell layers at P0 and is confined to the inner nuclear layer at P7
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| ♀ | phenotype observed in females |
| ♂ | phenotype observed in males |
| N | normal phenotype |
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• significant reduction in rod photoreceptors at P14, however no changes in the number of other major cell types in the retina
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• horizontal cell synaptogenesis in the retina is defective
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• significant reduction in rod photoreceptors at P14, however no changes in the number of other major cell types in the retina
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• horizontal cell synaptogenesis in the retina is defective
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| ♀ | phenotype observed in females |
| ♂ | phenotype observed in males |
| N | normal phenotype |
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• mice exhibit reduced thickness of the inner nuclear layer at P9
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• mice exhibit reduced thickness of the outer nuclear layer at P9
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• mice exhibit a reduction of retinal thickness by P15
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• mice exhibit early onset of retinal degeneration by P9-P15
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| ♀ | phenotype observed in females |
| ♂ | phenotype observed in males |
| N | normal phenotype |
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• only 10 - 25% of cells in dissociated retinal cell counts are rods compared to over 70% in wild-type mice
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• at E11.5 eyes are noticeably smaller compared to heterozygous littermates
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• lamination of the retina is severely reduced or absent
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• only 10 - 25% of cells in dissociated retinal cell counts are rods compared to over 70% in wild-type mice
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| ♀ | phenotype observed in females |
| ♂ | phenotype observed in males |
| N | normal phenotype |
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• more Muller cells are observed in the retina
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• more Muller cells are observed in the retina
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| ♀ | phenotype observed in females |
| ♂ | phenotype observed in males |
| N | normal phenotype |
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• mice hemizygous for the transgene are viable and exhibit no gross physical or behavioral abnormalities
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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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last database update 11/19/2024 MGI 6.24 |
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