Phenotypes associated with this allele

• Allele Symbol: Tg(Rho-icre)1Ck
• Allele Name: transgene insertion 1, Ching-Kang Chen
• Allele ID: MGI:3576662
• Summary: 11 genotypes

Jump to	Allelic Composition	Genetic Background	Genotype ID
cn1	Atrip^tm1.1Pof/Atrip^tm1.1Pof Tg(Rho-icre)1Ck/0	involves: 129P2/OlaHsd * C57BL/6 * SJL	MGI:6505487
cn2	Nampt^tm1Oleo/Nampt^tm1Oleo Tg(Rho-icre)1Ck/0	involves: 129P2/OlaHsd * C57BL/6 * SJL	MGI:6383809
cn3	Pdcl^tm1.1Bmw/Pdcl^tm1.1Bmw Tg(Rho-icre)1Ck/0	involves: 129S4/SvJaeSor * 129S6/SvEvTac * C57BL/6 * SJL	MGI:5516349
cn4	Ift172^tm1.1Rama/Ift172^tm1.1Rama Tg(Rho-icre)1Ck/0	involves: 129X1/SvJ * C57BL/6 * SJL	MGI:6383402
cn5	Emc1^em1Xjz/Emc1^em1Xjz Tg(Rho-icre)1Ck/0	involves: C57BL/6 * C57BL/6J * SJL	MGI:7611972
cn6	Nudc^tm1c(KOMP)Mbp/Nudc^tm1c(KOMP)Mbp Tg(Rho-icre)1Ck/0	involves: C57BL/6 * C57BL/6N * SJL	MGI:7618543
cn7	Arl3^Gt(EUCJ0159b07)1.1Hmgu/Arl3^Gt(EUCJ0159b07)1.1Hmgu Tg(Rho-icre)1Ck/0	involves: C57BL/6J * C57BL/6N * SJL	MGI:6386321
cn8	Nmnat1^tm1c(EUCOMM)Wtsi/Nmnat1^tm1c(EUCOMM)Wtsi Tg(Rho-icre)1Ck/0	involves: C57BL/6J * C57BL/6N * SJL	MGI:6272877
cn9	Nrf1^tm1c(KOMP)Wtsi/Nrf1^tm1c(KOMP)Wtsi Tg(Rho-icre)1Ck/0	involves: C57BL/6N * SJL	MGI:6383436
cn10	Elovl4^tm3Kzh/Elovl4^tm3Kzh Tg(Rho-icre)1Ck/?	involves: C57BL/6 * SJL	MGI:5492071
cn11	Cetn1^tm1Wbae/Cetn1^tm1Wbae Tg(Rho-icre)1Ck/0	involves: C57BL/6 * SJL	MGI:6361417

Genotype
MGI:6505487

cn1

• Allelic Composition: Atrip^tm1.1Pof/Atrip^tm1.1Pof; Tg(Rho-icre)1Ck/0
• Genetic Background: involves: 129P2/OlaHsd * C57BL/6 * SJL

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

vision/eye

vision/eye phenotype ( J:297493 )

N • retinas show normal lamination and outer nuclear layer and mice exhibit normal visual acuity at 7 months of age

Genotype
MGI:6383809

cn2

• Allelic Composition: Nampt^tm1Oleo/Nampt^tm1Oleo; Tg(Rho-icre)1Ck/0
• Genetic Background: involves: 129P2/OlaHsd * C57BL/6 * SJL

vision/eye

optic nerve atrophy ( J:265592 )

• secondary with pallor by 6 weeks

abnormal retina morphology ( J:265592 )

• rounded and constricted with loss of cristae in the retinal mitochondria by 4 weeks

• cytoplasmic vacuoles containing degenerating organelles including ruptured mitochondria

abnormal photoreceptor inner segment morphology ( J:265592 )

• by 4 weeks

abnormal photoreceptor outer segment morphology ( J:265592 )

• by 4 weeks

retina photoreceptor degeneration ( J:265592 )

• by 6 weeks with massive atrophy of the neurosensory retina and vascular attenuation with pigment mottling

• however, nicotinamide mononucleotide treatment rescued regeneration

retina pigment epithelium atrophy ( J:265592 )

• by 6 weeks

thin retina outer nuclear layer ( J:265592 )

• progressive and almost completely absent by 6 weeks

decreased total retina thickness ( J:265592 )

• by 6 weeks

abnormal cone electrophysiology ( J:265592 )

• reduced b-waves amplitudes by 6 weeks of age

• reduced visual acuity by 6 weeks of age

• however, nicotinamide mononucleotide treatment rescued function

abnormal rod electrophysiology ( J:265592 )

• reduced a-waves amplitudes by 6 weeks of age

• however, nicotinamide mononucleotide treatment rescued function

cellular

abnormal mitochondrial morphology ( J:265592 )

• rounded and constricted with loss of cristae in the retinal mitochondria by 4 weeks

abnormal mitochondrial crista morphology ( J:265592 )

• loss of cristae in the retinal mitochondria by 4 weeks

nervous system

abnormal photoreceptor inner segment morphology ( J:265592 )

• by 4 weeks

abnormal photoreceptor outer segment morphology ( J:265592 )

• by 4 weeks

retina photoreceptor degeneration ( J:265592 )

• by 6 weeks with massive atrophy of the neurosensory retina and vascular attenuation with pigment mottling

• however, nicotinamide mononucleotide treatment rescued regeneration

optic nerve atrophy ( J:265592 )

• secondary with pallor by 6 weeks

pigmentation

retina pigment epithelium atrophy ( J:265592 )

• by 6 weeks

Genotype
MGI:5516349

cn3

• Allelic Composition: Pdcl^tm1.1Bmw/Pdcl^tm1.1Bmw; Tg(Rho-icre)1Ck/0
• Genetic Background: involves: 129S4/SvJaeSor * 129S6/SvEvTac * C57BL/6 * SJL

vision/eye

abnormal retina photoreceptor morphology ( J:197148 )

short retina rod cell outer segment ( J:197148 )

• about 25% shortening of outer segments relative to controls at 35 days

• the very few rods remaining at 6 months are malformed with almost no outer segment

retina photoreceptor degeneration ( J:197148 )

• progressive photoreceptor degeneration

retina rod cell degeneration ( J:197148 )

• no loss at 1 month

• definite losses at 2 months

• half of nuclei are lost by 3-4 months

• almost all nuclei are lost by 9 months

abnormal eye physiology ( J:197148 )

photosensitivity ( J:197148 )

• reduced response to light flashes

• twelve fold increase in light intensity required for a 1/2 saturating response

abnormal electroretinogram waveform feature ( J:197148 )

• considerable reduction in light sensitivity and response amplitude

• seventeen fold desensitization for the a-wave and a two fold reduction in maximal response

• 25% decrease in response amplitude

• 3.8 fold reduction in signal amplification rate

• substantially decelerated falling phase

decreased visual acuity ( J:197148 )

• 3 fold reduction in visual acuity under dim lighting

• visual acuity is normal under bright lights

delayed dark adaptation ( J:197148 )

• recovery time constant is increased five fold

nervous system

abnormal retina photoreceptor morphology ( J:197148 )

short retina rod cell outer segment ( J:197148 )

• about 25% shortening of outer segments relative to controls at 35 days

• the very few rods remaining at 6 months are malformed with almost no outer segment

retina photoreceptor degeneration ( J:197148 )

• progressive photoreceptor degeneration

retina rod cell degeneration ( J:197148 )

• no loss at 1 month

• definite losses at 2 months

• half of nuclei are lost by 3-4 months

• almost all nuclei are lost by 9 months

homeostasis/metabolism

photosensitivity ( J:197148 )

• reduced response to light flashes

• twelve fold increase in light intensity required for a 1/2 saturating response

Genotype
MGI:6383402

cn4

• Allelic Composition: Ift172^tm1.1Rama/Ift172^tm1.1Rama; Tg(Rho-icre)1Ck/0
• Genetic Background: involves: 129X1/SvJ * C57BL/6 * SJL

vision/eye

abnormal photoreceptor outer segment morphology ( J:262800 )

abnormal photoreceptor outer segment disc membrane morphology ( J:262800 )

• outer segment disc disorganization and accumulation of extracellular debris is seen at P25 and P28

short photoreceptor outer segment ( J:262800 )

• mice show a shortening of the outer segment length at 1 month of age

disorganized photoreceptor outer segment ( J:262800 )

• mice show mislocalization of photoreceptor outer segment proteins

• at P31, the outer segments are disorganized and appear to lose the connection with the axoneme and innersegment of the photoreceptor cell

photoreceptor outer segment degeneration ( J:262800 )

• outer segments are fully degenerated by P31

retina outer nuclear layer degeneration ( J:262800 )

• mice show thinning of the outer nuclear layer at 1 month, with a 38% reduction, and a complete degeneration by 2 months

retina degeneration ( J:262800 )

• mice exhibit rapid retinal degeneration showing decreased neural retinal thickness due to thinning and eventual loss of the outer nuclear layer by 2 months of age

• however, mice do not show retinal cysts

abnormal electroretinogram waveform feature ( J:262800 )

• mixed rod/cone responses are reduced by half at 1 month of age

• by 2 months of age, the ERG is severely affected or undetectable across the stimulus conditions

decreased b-wave amplitude ( J:262800 )

• mice show an 84% reduction of rod-driven b-wave amplitude at 0.01 cd/m² light stimulus after dark adaptation at 1 month of age

• mice show a reduction in cone-isolated b-wave amplitude at 20 cd/m² light stimulus after light adaptation at 2 months of age, indicating secondary cone degeneration

nervous system

abnormal photoreceptor outer segment morphology ( J:262800 )

abnormal photoreceptor outer segment disc membrane morphology ( J:262800 )

• outer segment disc disorganization and accumulation of extracellular debris is seen at P25 and P28

short photoreceptor outer segment ( J:262800 )

• mice show a shortening of the outer segment length at 1 month of age

disorganized photoreceptor outer segment ( J:262800 )

• at P31, the outer segments are disorganized and appear to lose the connection with the axoneme and innersegment of the photoreceptor cell

• mice show mislocalization of photoreceptor outer segment proteins

photoreceptor outer segment degeneration ( J:262800 )

• outer segments are fully degenerated by P31

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
retinal degeneration		DOID:8466		J:262800

Genotype
MGI:7611972

cn5

• Allelic Composition: Emc1^em1Xjz/Emc1^em1Xjz; Tg(Rho-icre)1Ck/0
• Genetic Background: involves: C57BL/6 * C57BL/6J * SJL

vision/eye

increased retina apoptosis ( J:345827 )

• at 2 months of age mainly in the outer nuclear layer

thin retina outer nuclear layer ( J:345827 )

• at 2 months of age

abnormal retina photoreceptor layer morphology ( J:345827 )

• decreased thickness of the photoreceptor segment at 2 months of age

abnormal photoreceptor outer segment morphology ( J:345827 )

• mislocalized rhodopsin at 2 months of age

short photoreceptor outer segment ( J:345827 )

• at 2 months of age

disorganized photoreceptor outer segment ( J:345827 )

• at 2 months of age

photoreceptor outer segment degeneration ( J:345827 )

• most signs of degeneration at 2 months of age are seen in the outer segments

retina photoreceptor degeneration ( J:345827 )

• progressive degeneration with almost complete loss at 3.5 months of age

decreased a-wave amplitude ( J:345827 )

• at both the 3.0 and 10.0 cd s*m-2 flash intensity

decreased b-wave amplitude ( J:345827 )

• at both the 3.0 and 10.0 cd s*m-2 flash intensity

abnormal rod electrophysiology ( J:345827 )

• reduced scotopic response with both the amplitude of the a-wave and b-wave reduced at both the 3.0 and 10.0 cd s*m-2 flash intensity

nervous system

abnormal photoreceptor outer segment morphology ( J:345827 )

• mislocalized rhodopsin at 2 months of age

short photoreceptor outer segment ( J:345827 )

• at 2 months of age

disorganized photoreceptor outer segment ( J:345827 )

• at 2 months of age

photoreceptor outer segment degeneration ( J:345827 )

• most signs of degeneration at 2 months of age are seen in the outer segments

retina photoreceptor degeneration ( J:345827 )

• progressive degeneration with almost complete loss at 3.5 months of age

cellular

increased retina apoptosis ( J:345827 )

• at 2 months of age mainly in the outer nuclear layer

Genotype
MGI:7618543

cn6

• Allelic Composition: Nudc^{tm1c(KOMP)Mbp}/Nudc^{tm1c(KOMP)Mbp}; Tg(Rho-icre)1Ck/0
• Genetic Background: involves: C57BL/6 * C57BL/6N * SJL

vision/eye

increased retina apoptosis ( J:346299 )

• by 3 weeks of age, apoptosis in the outer nuclear layer is detected

abnormal photoreceptor inner segment morphology ( J:346299 )

• mitochondria within the inner segment are smaller and are mislocalized, being dispersed within the inner segment instead of being located against the plasma membrane

• rhodopsin is retained in the outer nuclear layer and inner segment in 2- and 3-week old mice, indicating that it is not normally transported to the outer segment

abnormal photoreceptor outer segment morphology ( J:346299 )

• loss of photoreceptors is concurrent with loss of rod outer segment proteins, including rhodopsin and transducin

abnormal photoreceptor outer segment disc membrane morphology ( J:346299 )

• rod photoreceptors show regions of abnormal disc overgrowth or occasional dysmorphic disc membranes

abnormal retina rod cell morphology ( J:346299 )

• rod cells frequently have an extended periciliary ridge and in some cases, the discs in the retina grow parallel to the axoneme

retina rod cell degeneration ( J:346299 )

• 40% loss of rods is seen at 3 weeks of age and at 6 weeks of age, rod photoreceptors are completely degenerated, with no detectable outer segment region

abnormal retina outer nuclear layer morphology ( J:346299 )

• rhodopsin is retained in the outer nuclear layer and inner segment in 2- and 3-week old mice, indicating that it is not normally transported to the outer segment

retina outer nuclear layer degeneration ( J:346299 )

• 40% loss of outer nuclear layer nuclei at 3 weeks of age, indicating early rod photoreceptor degeneration

decreased total retina thickness ( J:346299 )

• thinning retina at 3 weeks of age

• however, the remaining outer segments appear normal

abnormal electroretinogram waveform feature ( J:346299 )

decreased a-wave implicit time ( J:346299 )

• while a-wave implicit time is not affected at 3 weeks of age, by 6 weeks of age it is reduced in both the scotopic and photopic conditions

decreased a-wave amplitude ( J:346299 )

• scotopic a-wave amplitude is diminished and photopic a-wave amplitude is reduced at 3 weeks of age, however a-wave implicit times are not affected

• at 6 weeks of age, mice show no response in the scotopic condition and the a-wave is diminished and show a decrease in photopic a-wave amplitude

decreased b-wave amplitude ( J:346299 )

• scotopic b-wave amplitude is diminished and photopic b-wave amplitude is reduced at 3 weeks of age, however b-wave implicit times are not affected

• at 6 weeks of age, mice show no response in the scotopic condition and the b-wave is diminished and show a decrease in photopic a-wave amplitude

nervous system

increased microglial cell activation ( J:346299 )

• microglial activity is increased and microglia are also seen in the inner segment and in the retinal ganglion cell layer, while normal location of microglia is in the plexiform layers

• retinas show an increase in GFAP-positive Muller glial processes extending up through the retina

abnormal photoreceptor inner segment morphology ( J:346299 )

• mitochondria within the inner segment are smaller and are mislocalized, being dispersed within the inner segment instead of being located against the plasma membrane

• rhodopsin is retained in the outer nuclear layer and inner segment in 2- and 3-week old mice, indicating that it is not normally transported to the outer segment

abnormal photoreceptor outer segment morphology ( J:346299 )

• loss of photoreceptors is concurrent with loss of rod outer segment proteins, including rhodopsin and transducin

abnormal photoreceptor outer segment disc membrane morphology ( J:346299 )

• rod photoreceptors show regions of abnormal disc overgrowth or occasional dysmorphic disc membranes

abnormal retina rod cell morphology ( J:346299 )

• rod cells frequently have an extended periciliary ridge and in some cases, the discs in the retina grow parallel to the axoneme

retina rod cell degeneration ( J:346299 )

• 40% loss of rods is seen at 3 weeks of age and at 6 weeks of age, rod photoreceptors are completely degenerated, with no detectable outer segment region

cellular

increased retina apoptosis ( J:346299 )

• by 3 weeks of age, apoptosis in the outer nuclear layer is detected

increased microglial cell activation ( J:346299 )

• microglial activity is increased and microglia are also seen in the inner segment and in the retinal ganglion cell layer, while normal location of microglia is in the plexiform layers

• retinas show an increase in GFAP-positive Muller glial processes extending up through the retina

hematopoietic system

increased microglial cell activation ( J:346299 )

• microglial activity is increased and microglia are also seen in the inner segment and in the retinal ganglion cell layer, while normal location of microglia is in the plexiform layers

• retinas show an increase in GFAP-positive Muller glial processes extending up through the retina

immune system

increased microglial cell activation ( J:346299 )

• microglial activity is increased and microglia are also seen in the inner segment and in the retinal ganglion cell layer, while normal location of microglia is in the plexiform layers

• retinas show an increase in GFAP-positive Muller glial processes extending up through the retina

Genotype
MGI:6386321

cn7

• Allelic Composition: Arl3^{Gt(EUCJ0159b07)1.1Hmgu}/Arl3^{Gt(EUCJ0159b07)1.1Hmgu}; Tg(Rho-icre)1Ck/0
• Genetic Background: involves: C57BL/6J * C57BL/6N * SJL

vision/eye

abnormal retina rod cell outer segment morphology ( J:232436 )

• slightly reduced

retina cone cell degeneration ( J:232436 )

• at 2 months

retina rod cell degeneration ( J:232436 )

• at 2 months

thin retina inner nuclear layer ( J:232436 )

• slightly

decreased total retina thickness ( J:232436 )

• progressive, at P15 and further by P50

abnormal cone electrophysiology ( J:232436 )

• begins to decline at 1 month

abnormal rod electrophysiology ( J:232436 )

• reduced a- and b-waves at 1 month

• however, readings at P15 are normal

behavior/neurological

decreased optokinetic reflex ( J:232436 )

• extinct at 2 months

• however, optokinetic response at 1 month is normal

nervous system

abnormal retina rod cell outer segment morphology ( J:232436 )

• slightly reduced

retina cone cell degeneration ( J:232436 )

• at 2 months

retina rod cell degeneration ( J:232436 )

• at 2 months

Genotype
MGI:6272877

cn8

• Allelic Composition: Nmnat1^{tm1c(EUCOMM)Wtsi}/Nmnat1^{tm1c(EUCOMM)Wtsi}; Tg(Rho-icre)1Ck/0
• Genetic Background: involves: C57BL/6J * C57BL/6N * SJL

nervous system

abnormal retina rod cell outer segment morphology ( J:267630 )

• no rod outer segments are detectable by P15

retina rod cell degeneration ( J:267630 )

• the outer nuclear layer is reduced at P28 but not P7, indicating that most rods are lost

vision/eye

abnormal retina rod cell outer segment morphology ( J:267630 )

• no rod outer segments are detectable by P15

retina rod cell degeneration ( J:267630 )

• the outer nuclear layer is reduced at P28 but not P7, indicating that most rods are lost

retina outer nuclear layer degeneration ( J:267630 )

• the outer nuclear layer is reduced at P28 but not P7, indicating that most rods are lost

retina degeneration ( J:267630 )

Genotype
MGI:6383436

cn9

• Allelic Composition: Nrf1^{tm1c(KOMP)Wtsi}/Nrf1^{tm1c(KOMP)Wtsi}; Tg(Rho-icre)1Ck/0
• Genetic Background: involves: C57BL/6N * SJL

vision/eye

decreased retina cone cell number ( J:267504 )

• at 8 weeks of age

decreased retina rod cell number ( J:267504 )

• at 8 weeks of age

short photoreceptor inner segment ( J:267504 )

• at 6 weeks of age

short photoreceptor outer segment ( J:267504 )

• at 6 weeks of age

retina rod cell degeneration ( J:267504 )

thin retina outer nuclear layer ( J:267504 )

• starting at 8 weeks of age and almost disappeared at 5 months

abnormal cone electrophysiology ( J:267504 )

• b-wave amplitudes decline at 8 weeks and are undetectable beyond 10 weeks of age

abnormal rod electrophysiology ( J:267504 )

• a-wave amplitudes decline at 6 weeks and are completely diminished at 3 months of age

cellular

abnormal mitochondrial morphology ( J:267504 )

• more rounded shape in rod cells with clustering of mitochondria near the outer limiting membrane unlike in wild-type cells

decreased mitochondrial size ( J:267504 )

• in rod cells

increased mitochondrial number ( J:267504 )

• 2.5 times in rod cells of the inner segment

abnormal mitochondrial physiology ( J:267504 )

• reduced COX activity in rod cells of the inner segment

nervous system

decreased retina cone cell number ( J:267504 )

• at 8 weeks of age

decreased retina rod cell number ( J:267504 )

• at 8 weeks of age

short photoreceptor inner segment ( J:267504 )

• at 6 weeks of age

short photoreceptor outer segment ( J:267504 )

• at 6 weeks of age

retina rod cell degeneration ( J:267504 )

Genotype
MGI:5492071

cn10

• Allelic Composition: Elovl4^tm3Kzh/Elovl4^tm3Kzh; Tg(Rho-icre)1Ck/?
• Genetic Background: involves: C57BL/6 * SJL

vision/eye

vision/eye phenotype ( J:194246 )

N • no abnormalities

N • normal ERG and normal visual performance up to 6.5 months

abnormal retina rod cell morphology ( J:194246 )

• statistically significant decrease in very long chain polyunsaturated fatty acid content of rods

homeostasis/metabolism

decreased unsaturated fatty acids level ( J:194246 )

• statistically significant decrease in very long chain polyunsaturated fatty acid content of rods

• decreases by 97.6%

nervous system

abnormal retina rod cell morphology ( J:194246 )

• statistically significant decrease in very long chain polyunsaturated fatty acid content of rods

Genotype
MGI:6361417

cn11

• Allelic Composition: Cetn1^tm1Wbae/Cetn1^tm1Wbae; Tg(Rho-icre)1Ck/0
• Genetic Background: involves: C57BL/6 * SJL

vision/eye

vision/eye phenotype ( J:276829 )

N • electroretinograms show no significant differences in scotopic ERG a-wave amplitudes relative to controls