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Phenotypes associated with this allele
Allele Symbol
Allele Name
Allele ID
Unc5crcmTg(Ucp)1.23Kz
rostral cerebellar malformation (Tg(Ucp)1.23Kz)
MGI:1857852
Summary 4 genotypes
Jump to Allelic Composition Genetic Background Genotype ID
hm1
Unc5crcmTg(Ucp)1.23Kz/Unc5crcmTg(Ucp)1.23Kz B6.Cg-Unc5crcmTg(Ucp)1.23Kz MGI:3665464
hm2
Unc5crcmTg(Ucp)1.23Kz/Unc5crcmTg(Ucp)1.23Kz involves: C57BL/6J * SJL/J MGI:2446453
cx3
Dcctm1Wbg/Dcc+
Unc5crcmTg(Ucp)1.23Kz/Unc5crcmTg(Ucp)1.23Kz
involves: 129X1/SvJ * C57BL/6J * SJL/J MGI:3665460
cx4
Dcctm1Wbg/Dcc+
Unc5crcmTg(Ucp)1.23Kz/Unc5c+
involves: 129X1/SvJ * C57BL/6J * SJL/J MGI:3665461


Genotype
MGI:3665464
hm1
Allelic
Composition
Unc5crcmTg(Ucp)1.23Kz/Unc5crcmTg(Ucp)1.23Kz
Genetic
Background
B6.Cg-Unc5crcmTg(Ucp)1.23Kz
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Unc5crcmTg(Ucp)1.23Kz mutation (0 available); any Unc5c mutation (61 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• Background Sensitivity: a decrease in number surviving to weaning compared to homozygotes on a mixed C57BL/6J and SJL/J background is seen after 3 generations of backcrosses
• after 10 generations of backcrosses to C57BL/6J, no mice survive to weaning

nervous system
• the trochlear nerve projects ventrally rather than dorsally and some of the axons exit the CNS ipsilaterally rather than contralaterally
• Background Sensitivity: all mice have abnormalities in at least 1 trochlear nerve on an inbred C57BL/6J background compared to only 2 of 5 mice on a mixed C57BL/6J and SJL/J background
• reduced diameter proximal to the point of contact with the diaphragm
• reduction in diameter correlates to the reduction in diaphragm innervation
• the number of axons in the phrenic nerve is significantly reduced
• at E18.5 from 30 to 75% of the diaphragm is uninnervated by motor neurons
• absence of innervation ranges from complete and unilateral to partial and bilateral
• however, motor neuron innervation of intercostal muscles and sensory neuron innervation around the lateral edge of the diaphragm appear normal
• areas lacking innervation do not appear to have ever been in contact with motor neurons
• Background Sensitivity: penetrance and severity of the defect in diaphragm motor neuron innervation are increased on an inbred C57BL/6J background compared to a mixed C57BL/6J and SJL/J background




Genotype
MGI:2446453
hm2
Allelic
Composition
Unc5crcmTg(Ucp)1.23Kz/Unc5crcmTg(Ucp)1.23Kz
Genetic
Background
involves: C57BL/6J * SJL/J
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Unc5crcmTg(Ucp)1.23Kz mutation (0 available); any Unc5c mutation (61 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
N
• Background Sensitivity: no difference from the expected number of homozygotes is detected at weaning unlike in mice crossed for 10 generations to C57BL/6J where no homozygotes survive to weaning

behavior/neurological

nervous system
• an abnormal band of granule cells extends from the cerebellum into the inferior colliculus (J:40243)
• partial fusion of the inferior colliculus to the rostral cerebellum (J:40243)
• ectopic subpial cell populations identical to those in Unc5crcm homozygotes are seen (J:45577)
• only 5 lobes are formed
• vermal fissure formation is also abnormal in the lateral cerebral hemisphere
• a reduction in size and number of folia is seen in midline sagital sections
• abnormal external granule cell migration with cells migrating in cohorts rather than as single cells
• these abnormal groups of cells are often associated with gaps in the Purkinje cell layer
• the pyramidal decussation is thinner but present
• an abnormal band of granule cells extends from the cerebellum into the inferior colliculus
• partial fusion of the inferior colliculus to the rostral cerebellum with ectopic granule and Purkinje cells in the inferior colliculus and tegmentum of the midbrain
• abnormal groups of migrating granule cells are associated with gaps in the Purkinje cell layer
• in the inferior colliculus and tegmentum of the midbrain (J:40243)
• in the inferior colliculus and pontine area at P7 (J:45577)
• an abnormal band of granule cells extends from the cerebellum into the inferior colliculus (J:40243)
• in the inferior colliculus and pontine area at P7 (J:45577)
• more pronounced than in Unc5crcm homozygotes
• Background Sensitivity: penetrance and severity of the defect in diaphragm motor neuron innervation are decreased on a mixed mixed C57BL/6J and SJL/J background compared to an inbred C57BL/6J background
• expression analysis indicates the absence of the corticospinal tract in the dorsal funiculus
• just before the decussation the pyramidal tract broadens and at the decussation 2 bundles of axons form with some but not all axons crossing the midline
• most axons that cross the midline fail to enter the dorsal funiculus and are instead found in the adjacent dorsal gray matter and just into the white matter of the contralateral lateral funiculus of the cervical and thoracic spinal cord
• axons that fail to cross the midline follow an ipsilateral path into the lateral funiculus
• Background Sensitivity: only 2 of 5mice have abnormalities in at least 1 trochlear nerve on a mixed C57BL/6J and SJL/J background while abnormalities are seen in all mice on an inbred C57BL/6J background
• the dorsal funiculus is smaller and its ventral aspect appears shorter and thinner
• expression analysis indicates the absence of the corticospinal tract in the dorsal funiculus




Genotype
MGI:3665460
cx3
Allelic
Composition
Dcctm1Wbg/Dcc+
Unc5crcmTg(Ucp)1.23Kz/Unc5crcmTg(Ucp)1.23Kz
Genetic
Background
involves: 129X1/SvJ * C57BL/6J * SJL/J
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Dcctm1Wbg mutation (1 available); any Dcc mutation (76 available)
Unc5crcmTg(Ucp)1.23Kz mutation (0 available); any Unc5c mutation (61 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
nervous system
• some axons fail to cross the midline at the pyramidal decussation and instead extend into the lateral and ventral funiculus

cellular
• some axons fail to cross the midline at the pyramidal decussation and instead extend into the lateral and ventral funiculus




Genotype
MGI:3665461
cx4
Allelic
Composition
Dcctm1Wbg/Dcc+
Unc5crcmTg(Ucp)1.23Kz/Unc5c+
Genetic
Background
involves: 129X1/SvJ * C57BL/6J * SJL/J
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Dcctm1Wbg mutation (1 available); any Dcc mutation (76 available)
Unc5crcmTg(Ucp)1.23Kz mutation (0 available); any Unc5c mutation (61 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
nervous system
• a few axons fail to cross the midline at the pyramidal decussation and instead extend into the ventral funiculus of the cervical spinal cord

cellular
• a few axons fail to cross the midline at the pyramidal decussation and instead extend into the ventral funiculus of the cervical spinal cord





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last database update
04/30/2024
MGI 6.23
The Jackson Laboratory