Phenotypes associated with this allele

• Allele Symbol: Reln^rl
• Allele Name: reeler
• Allele ID: MGI:1856398
• Summary: 15 genotypes

Jump to	Allelic Composition	Genetic Background	Genotype ID
hm1	Reln^rl/Reln^rl	B6.Cg-Reln^rl	MGI:3640191
hm2	Reln^rl/Reln^rl	C3.Cg-Reln^rl	MGI:3640190
hm3	Reln^rl/Reln^rl	involves: 129S4/SvJaeSor * C57BL/6J	MGI:2654921
hm4	Reln^rl/Reln^rl	involves: BALB/c	MGI:4835177
hm5	Reln^rl/Reln^rl	involves: C3HeB/FeJ * C57BL/6J	MGI:3514040
hm6	Reln^rl/Reln^rl	Not Specified	MGI:2684661
ht7	Reln^rl/Reln^+	B6C3Fe a/a-Reln^rl/J	MGI:5661067
ht8	Reln^rl/Reln^+	B6.Cg-Reln^rl	MGI:5689835
ht9	Reln^rl/Reln^+	involves: BALB/c	MGI:3720601
cn10	Gt(ROSA)26Sor^tm1(DTA)Riet/Gt(ROSA)26Sor^+ Reln^rl/Reln^+ Trp73^tm1(cre)Agof/Trp73^+	involves: 129P2/OlaHsd * 129S1/Sv * 129X1/SvJ	MGI:4365610
cn11	Gt(ROSA)26Sor^tm1(DTA)Riet/Gt(ROSA)26Sor^+ Reln^rl/Reln^+ Wnt3a^tm1(cre)Eag/Wnt3a^+	involves: 129P2/OlaHsd * 129S1/Sv * 129X1/SvJ	MGI:4365611
cn12	Gt(ROSA)26Sor^tm1(DTA)Riet/Gt(ROSA)26Sor^+ Reln^rl/Reln^+ Trp73^tm1(cre)Agof/Trp73^+ Wnt3a^tm1(cre)Eag/Wnt3a^+	involves: 129P2/OlaHsd * 129S1/Sv * 129X1/SvJ	MGI:4365612
cx13	Dab1^tm3.1Cpr/Dab1^tm3.1Cpr Reln^rl/Reln^+	involves: 129S4/SvJaeSor * C57BL/6J	MGI:3810296
cx14	Dab1^tm1Cpr/Dab1^tm4Cpr Reln^rl/Reln^+	involves: 129/Sv * C3HeB/Fe * C57BL/6	MGI:3050790
cx15	Reln^rl/Reln^rl Tg(Mbp-MAPT/lacZ)#aCaw/Tg(Mbp-MAPT/lacZ)#aCaw	involves: BALB/c * C57BL/6 * C57BL/6J	MGI:4889081

Genotype
MGI:3640191

hm1

• Allelic Composition: Reln^rl/Reln^rl
• Genetic Background: B6.Cg-Reln^rl

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

mortality/aging

premature death ( J:5312 )

• Background Sensitivity: all die by 30 days of age, earlier than on a C3H/HeJ background

behavior/neurological

impaired righting response ( J:5312 )

• Background Sensitivity: if turned on the side, mutants on a C57BL/6J background kick all legs futilely in unison and may not be able to right themselves without assistance, a phenotype not seen on the C3H/HeJ background

tremors ( J:5312 )

• Background Sensitivity: mice show rapid unsustained low amplitude tremor of the body when walking starting at P13 that is not readily observed on a C3H/HeJ background

ataxia ( J:5312 )

• Background Sensitivity: mice show ataxic gait beginning at P13 that is more severe than on a C3H/HeJ background

abnormal gait ( J:5312 )

• hind legs tend to splay and mice frequently fall on their sides

decreased locomotor activity ( J:5312 )

• Background Sensitivity: general level of activity begins to decrease during the third week of life unlike on a C3H/HeJ background in which activity is normal

growth/size/body

decreased body weight ( J:5312 )

• Background Sensitivity: mice attain maximum weight by the end of the second week of life, then growth ceases during the third week and mutants remain smaller thereafter

postnatal growth retardation ( J:5312 )

• Background Sensitivity: growth retardation is more severe than on a C3H/HeJ background

reproductive system

infertility ( J:5312 )

• Background Sensitivity: mutants with a C57BL/6J background do not live long enough to breed while those with a C3H/HeJ background may be fertile

nervous system

abnormal entorhinal cortex morphology ( J:5312 )

• the entorhinal cortex exhibits an outer zone of tangentially oriented polymorphic cells in the superficial plane normally given to an external plexiform layer

• a wide inner zone of larger cells in the entorhinal cortex is cytologically similar to the outer large cell layers of the normal

abnormal hippocampus morphology ( J:5312 )

abnormal hippocampus CA1 region morphology ( J:5312 )

• CA1 has two separate cellular laminae

abnormal hippocampus CA2 region morphology ( J:5312 )

• CA2 is subluxed away from its junction with CA1

abnormal dentate gyrus morphology ( J:5312 )

• many granule cells of the dentate gyrus are scattered through the hilus and are intermixed with large cells of CA4

abnormal olfactory cortex morphology ( J:5312 )

• the piriform cortex is composed of an outer polymorphic and inner large cell zone, resembling the deep polymorphic and overlying pyramidal zones of wild-type

• immediately subjacent to the lateral olfactory tract, the outer polymorphic zone is attenuated

abnormal sensory neuron innervation pattern ( J:5312 )

• fiber bundles course from the olfactory crus in aberrant superficial relation to the lateral olfactory tract and join the main mass of the anterior commissure at a more caudal level

Genotype
MGI:3640190

hm2

• Allelic Composition: Reln^rl/Reln^rl
• Genetic Background: C3.Cg-Reln^rl

mortality/aging

premature death ( J:5312 )

• Background Sensitivity: 80% die by 40 days of age, the rest survive and are in normal health, indicating increased vitality than on a C57BL/6J background

behavior/neurological

behavior/neurological phenotype ( J:5312 )

N • Background Sensitivity: mutants on a C57BL/6J background crossed to C3H/HeJ to the N7 generation only show mild behavioral/neurological disabilities compared to mutants on a C57BL/6J background, and are able to right themselves and remain active

ataxia ( J:5312 )

• Background Sensitivity: at around 4 weeks of age, develop a more moderate ataxia during ambulation than on a C57BL/6J background

growth/size/body

postnatal growth retardation ( J:5312 )

• Background Sensitivity: growth retardation is much less severe than on a C57BL/6J background

slow postnatal weight gain ( J:5312 )

• Background Sensitivity: exhibit normal weight gain during the first 2 weeks of life, then growth decreases in the third week but weight gain resumes after weaning, although at a slower rate so that by 60 days, they are still growing but weight is less than 70% of wild-type

digestive/alimentary system

diarrhea ( J:5312 )

• more than half of the mutants lost during the 20- to 30-day interval die as a consequence of diarrhea

nervous system

abnormal neuronal migration ( J:12728 )

• different classes of neurons take their orgin from the ependymal layer at the normal time but migrate abnormally and come to rest in abnormal relations to each other

• migratory ascent along the radial glial fibers (RGF) proceeds normally as the cell crosses the IZ but is blocked upon encounter with postmigratory neurons within the cortex

abnormal entorhinal cortex morphology ( J:5312 )

• the entorhinal cortex exhibits an outer zone of tangentially oriented polymorphic cells in the superficial plane normally given to an external plexiform layer

• a wide inner zone of larger cells in the entorhinal cortex is cytologically similar to the outer large cell layers of the normal

abnormal hippocampus morphology ( J:5312 )

abnormal hippocampus CA1 region morphology ( J:5312 )

• CA1 has two separate cellular laminae

abnormal hippocampus CA2 region morphology ( J:5312 )

• CA2 is subluxed away from its junction with CA1

abnormal dentate gyrus morphology ( J:5312 )

• many granule cells of the dentate gyrus are scattered through the hilus and are intermixed with large cells of CA4

abnormal cerebral cortex morphology ( J:12728 )

• the ascending glial fiber gives rise to 3 or less terminal branches (compared to 3-5 or more in wild-type) within the superplate (SP) rather than the pleriform zone (PZ)

• at the lower margin of the cortex, cells are frequently found bunched close one upon the other with substantial overlap of radially adjacent cells and the leading processes of these cells are abnormally short and blunt

• extent of contact between the somatic surfaces of postmigratory neurons and the surfaces of the radial glial fibers is substantially greater than in wild-type indicating abnormal adhesions between the postmigratory cells and the radial glial fibers

abnormal stratification in cerebral cortex ( J:12728 )

abnormal olfactory cortex morphology ( J:5312 )

• the piriform cortex is composed of an outer polymorphic and inner large cell zone, resembling the deep polymorphic and overlying pyramidal zones of wild-type

• immediately subjacent to the lateral olfactory tract, the outer polymorphic zone is attenuated

abnormal sensory neuron innervation pattern ( J:5312 )

• fiber bundles course from the olfactory crus in aberrant superficial relation to the lateral olfactory tract and join the main mass of the anterior commissure at a more caudal level

cellular

abnormal neuronal migration ( J:12728 )

• different classes of neurons take their orgin from the ependymal layer at the normal time but migrate abnormally and come to rest in abnormal relations to each other

• migratory ascent along the radial glial fibers (RGF) proceeds normally as the cell crosses the IZ but is blocked upon encounter with postmigratory neurons within the cortex

Genotype
MGI:2654921

hm3

• Allelic Composition: Reln^rl/Reln^rl
• Genetic Background: involves: 129S4/SvJaeSor * C57BL/6J

nervous system

abnormal cortical marginal zone morphology ( J:74239 )

• the neocortical marginal zone is crowded with polymorphic cells unlike in wild-type mice

abnormal dentate gyrus morphology ( J:74239 )

• the dentate gyrus is disrupted

abnormal hippocampus pyramidal cell layer ( J:74239 )

• the pyramidal cells are scattered over a broad region unlike in wild-type mice

Genotype
MGI:4835177

hm4

• Allelic Composition: Reln^rl/Reln^rl
• Genetic Background: involves: BALB/c

behavior/neurological

abnormal sexual interaction ( J:14535 )

• vestibular stimulation improves the ability of females to mate with experienced males

• vestibular stimulation improves the ability of males to mate with experienced females

Genotype
MGI:3514040

hm5

• Allelic Composition: Reln^rl/Reln^rl
• Genetic Background: involves: C3HeB/FeJ * C57BL/6J

nervous system

abnormal neuron differentiation ( J:12728 )

• abnormal arrangement of neurons is seen in brain structures

abnormal cerebral cortex morphology ( J:12728 )

• autoradiographic studies of development of the cerebral cortex have shown that the different classes of neurons originate from the ependymal layer at the normal time but migrate abnormally in relation to each other

abnormal cerebellum morphology ( J:12728 )

• typical organization and lamination of the cerebellar cortex, the cerebral cortex, and the hippocampus are affected

small cerebellum ( J:12728 )

cellular

abnormal neuron differentiation ( J:12728 )

• abnormal arrangement of neurons is seen in brain structures

Genotype
MGI:2684661

hm6

• Allelic Composition: Reln^rl/Reln^rl
• Genetic Background: Not Specified

mortality/aging

lethality at weaning, complete penetrance ( J:13038 )

• many die at around 3 weeks of age, although delaying weaning and providing moist food prolongs life

growth/size/body

decreased body size ( J:13038 )

• often smaller at around 15 days of age

postnatal growth retardation ( J:13038 )

behavior/neurological

tremors ( J:13038 )

• a slight tremor of the foot is usually seen when the mutants fall and a more generalized tremor is sometimes seen when the mutant is excited and active

impaired balance ( J:13038 )

• unable to keep hindquaters upright and when walking or running, frequently fall over on their sides

abnormal gait ( J:13038 )

• when standing still, hindquaters sway slowly from side to side and when walking, swaying is accentuated and the mutant falls over on its side, however righting is easily achieved

• exhibit some improvement with age as some adults are able to run without falling over, however legs are kept further apart

decreased locomotor activity ( J:13038 )

• reduction in activity is identifiable at 15 days of age

reproductive system

reduced female fertility ( J:13038 )

♀ • majority of females are sterile

male infertility ( J:13038 )

♂

integument

disheveled coat ( J:13038 )

• fur of adults exhibits an unkempt appearance

Genotype
MGI:5661067

ht7

• Allelic Composition: Reln^rl/Reln⁺
• Genetic Background: B6C3Fe a/a-Reln^rl/J

nervous system

abnormal ventral striatum morphology ( J:107996 )

• mice show an elevation of dopamine D3 receptor expression in the ventral striatum, particularly in the nucleus accumbens and tuberculum olfactorium

decreased dopaminergic neuron number ( J:107996 )

• mice exhibit a reduction in the number of tyrosine hydroxylase-immunoreactive cell bodies in the ventral tegmental area, but not in the medial substantia nigra, pars compacta, indicating dopamine cell loss in the ventral striatum

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
schizophrenia		DOID:5419	OMIM:181500	J:107996

Genotype
MGI:5689835

ht8

• Allelic Composition: Reln^rl/Reln⁺
• Genetic Background: B6.Cg-Reln^rl

behavior/neurological

abnormal short-term spatial reference memory ( J:223944 )

• chronic administration of corticosterone to induce high stress hormone levels results in disrupted short-term spatial memory in both males and females as indicated by mice showing no preference for the novel arm of the Y-maze as seen in controls

• however, unstressed mutants show normal spatial memory on the Y-maze

abnormal social investigation ( J:223944 )

♂ • untreated and corticosterone treated (stressed) males spend less time sniffing a novel mouse during phase 2 of the social recognition test when mice can choose between a novel and a familiar mouse, indicating impaired social recognition memory

nervous system

abnormal prepulse inhibition ( J:223944 )

♂ • corticosterone treated males do not exhibit disrupted prepulse inhibition as is seen in wild-type corticosterone treated males, indicating that males are resilient to the effects of stress on prepulse inhibition

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
schizophrenia		DOID:5419	OMIM:181500	J:223944

Genotype
MGI:3720601

ht9

• Allelic Composition: Reln^rl/Reln⁺
• Genetic Background: involves: BALB/c

nervous system

nervous system phenotype ( J:42417 )

N • cerebellum is normally foliated and qualitatively indistinguishable from that of wild-type controls

Purkinje cell degeneration ( J:42417 )

• males, but not females, have a 16% reduction in the number of Purkinje cells at 3 months of age and a 24% reduction at 16 months of age with the loss occurring throughout the mediolateral extent of the cerebellum

decreased Purkinje cell number ( J:42417 )

small cerebellum ( J:42417 )

• males, but not females, have reduction in overall cross-sectional area of the cerebella at 3 and 16 months of age

Genotype
MGI:4365610

cn10

• Allelic Composition: Gt(ROSA)26Sor^tm1(DTA)Riet/Gt(ROSA)26Sor⁺; Reln^rl/Reln⁺; Trp73^tm1(cre)Agof/Trp73⁺
• Genetic Background: involves: 129P2/OlaHsd * 129S1/Sv * 129X1/SvJ

nervous system

decreased Cajal-Retzius cell number ( J:153209 )

• number of Cajal-Retzius cells in the cortical marginal zone is reduced by 72% at P0

Genotype
MGI:4365611

cn11

• Allelic Composition: Gt(ROSA)26Sor^tm1(DTA)Riet/Gt(ROSA)26Sor⁺; Reln^rl/Reln⁺; Wnt3a^tm1(cre)Eag/Wnt3a⁺
• Genetic Background: involves: 129P2/OlaHsd * 129S1/Sv * 129X1/SvJ

nervous system

decreased Cajal-Retzius cell number ( J:153209 )

• number of Cajal-Retzius cells in the cortical marginal zone is reduced by 35% at P0

Genotype
MGI:4365612

cn12

• Allelic Composition: Gt(ROSA)26Sor^tm1(DTA)Riet/Gt(ROSA)26Sor⁺; Reln^rl/Reln⁺; Trp73^tm1(cre)Agof/Trp73⁺; Wnt3a^tm1(cre)Eag/Wnt3a⁺
• Genetic Background: involves: 129P2/OlaHsd * 129S1/Sv * 129X1/SvJ

nervous system

decreased Cajal-Retzius cell number ( J:153209 )

• number of Cajal-Retzius cells (CRc) in the cortical marginal zone is reduced by 84% at P0

• nearly all CRc in the hippocampal formation are ablated

Genotype
MGI:3810296

cx13

• Allelic Composition: Dab1^tm3.1Cpr/Dab1^tm3.1Cpr; Reln^rl/Reln⁺
• Genetic Background: involves: 129S4/SvJaeSor * C57BL/6J

nervous system

nervous system phenotype ( J:74239 )

N • the hippocampus and neocortex are normal

Genotype
MGI:3050790

cx14

• Allelic Composition: Dab1^tm1Cpr/Dab1^tm4Cpr; Reln^rl/Reln⁺
• Genetic Background: involves: 129/Sv * C3HeB/Fe * C57BL/6

nervous system

abnormal cortical marginal zone morphology ( J:91679 )

• the marginal zone of the cortex is reduced, similar to compound heterozygous mice wild-type for Reln

abnormal hippocampus morphology ( J:91679 )

• the hippocampus is subtly disorganized in the CA2/3 region compared to mice heterozygous for Dab1^tm1Cpr and Reln^rl

Genotype
MGI:4889081

cx15

• Allelic Composition: Reln^rl/Reln^rl; Tg(Mbp-MAPT/lacZ)#aCaw/Tg(Mbp-MAPT/lacZ)#aCaw
• Genetic Background: involves: BALB/c * C57BL/6 * C57BL/6J

nervous system

abnormal axon extension ( J:168258 )

• thalamocortical axons fail to extend beneath the cortical plate and grow obliquely across it unlike in wild-type mice

cellular

abnormal axon extension ( J:168258 )

• thalamocortical axons fail to extend beneath the cortical plate and grow obliquely across it unlike in wild-type mice