All Phenotypes Mbp<shi> MGI Mouse

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Phenotypes associated with this allele

Allele Symbol
Allele Name
Allele ID

Mbp^shi
shiverer
MGI:1856159

Summary

12 genotypes

Jump to	Allelic Composition	Genetic Background	Genotype ID
hm1	Mbp^shi/Mbp^shi	C3Fe.SWV-Mbp^shi/J	MGI:3698853
hm2	Mbp^shi/Mbp^shi	involves: 129S1/Sv * 129X1/SvJ * C57BL/6J * SWV	MGI:3620244
hm3	Mbp^shi/Mbp^shi	involves: BALB/c * C3HeB/Fe * SWV	MGI:4834575
hm4	Mbp^shi/Mbp^shi	involves: C3H * ICR * SWV	MGI:4834528
hm5	Mbp^shi/Mbp^shi	involves: SWV	MGI:4834543
hm6	Mbp^shi/Mbp^shi	SWV-Mbp^shi	MGI:4834527
ht7	Mbp^shi/Mbp⁺	C3Fe.SWV-Mbp^shi/J	MGI:3698748
cx8	Mbp^shi/Mbp^shi Plp1^tm1Kan/Y	involves: 129S1/Sv * 129X1/SvJ * C57BL/6J * SWV	MGI:3620243
cx9	Mbp^shi/Mbp^shi Plp1^tm1Kan/Y	involves: 129S1/Sv * 129X1/SvJ * SWV	MGI:3620245
cx10	Mbp^shi/Mbp⁺ Mobp^tm1Irg/Mobp⁺	involves: 129S1/SvImJ * C57BL/6J * SWV	MGI:3613511
cx11	Mbp^shi/Mbp^shi Tg(Thy1-YFP)HJrs/0	involves: C3HeB/Fe * C57BL/6 * CBA * SWV	MGI:4834581
cx12	Mbp^shi/Mbp^shi Pmp2^{tm1.1(KOMP)Vlcg}/Pmp2^{tm1.1(KOMP)Vlcg}	involves: C3HeB/FeJ * C57BL/6 * C57BL/6NTac	MGI:5803708

Allelic Composition	Mbp^shi/Mbp^shi
Genetic Background	C3Fe.SWV-Mbp^shi/J

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Mbp^shi mutation (3 available); any Mbp mutation (32 available)

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

The Mbp^shi/Mbp^shi mouse

behavior/neurological

limb grasping ( J:211344 )

• mice show severe hind limb clasping at P56

tremors ( J:211344 )

• mice develop a severe generalized tremor during locomotion by 2 weeks of age, indicating CNS defects

hearing/vestibular/ear

reduced linear vestibular evoked potential ( J:116914 )

• elevated threshold for aged mice and prolonged latency for central response peaks in young and aged mice

immune system

immune system phenotype ( J:41447 )

N	• T cell, B cell, and macrophage counts are normal

decreased susceptibility to Picornaviridae infection ( J:41447 )

• following infection with Theiler's murine encephalomyelitis virus (TMEV), mice exhibit no viral RNA or signs of infection in the spinal cord unlike similarly treated wild-type mice

nervous system

abnormal myelin sheath morphology ( J:211344 )

• mice show a significant increase in the number of Schmidt-Lanterman incisures on sciatic nerve cross sections relative to control mice

• however, no major structural defects in PNS myelination are detected at P10 or P56 and the distribution of Na_v- and K_v1.2-channels in teased sciatic nerve fibers is normal at P56, indicating that the structure of the node of Ranvier is preserved

decreased nerve conduction velocity ( J:211344 )

• at P56, mice show a significant reduction in motor nerve conduction velocity relative to control mice

Allelic Composition	Mbp^shi/Mbp^shi
Genetic Background	involves: 129S1/Sv * 129X1/SvJ * C57BL/6J * SWV

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Mbp^shi mutation (3 available); any Mbp mutation (32 available)

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

mortality/aging

premature death ( J:38856 )

• die around 3 months of age

nervous system

abnormal myelin sheath morphology ( J:38856 )

• when present axon sheaths are thinner than normal and poorly compacted

abnormal myelination ( J:38856 )

• dysmyelination with many naked axons

behavior/neurological

tremors ( J:38856 )

ataxia ( J:38856 )

Allelic Composition	Mbp^shi/Mbp^shi
Genetic Background	involves: BALB/c * C3HeB/Fe * SWV

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Mbp^shi mutation (3 available); any Mbp mutation (32 available)

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

immune system

increased T cell proliferation ( J:48412 )

• T cells primed with MBP proliferate in culture unlike wild-type cells

increased interferon-gamma secretion ( J:48412 )

• when primed with MBP

increased interleukin-2 secretion ( J:48412 )

• when primed with MBP

increased interleukin-4 secretion ( J:48412 )

• when primed with MBP

increased susceptibility to experimental autoimmune encephalomyelitis ( J:48412 )

• following induction of experimental autoimmune encephalomyelitis (EAE), mice develop moderate to severe EAE unlike similarly treated wild-type mice

hematopoietic system

increased T cell proliferation ( J:48412 )

• T cells primed with MBP proliferate in culture unlike wild-type cells

cellular

increased T cell proliferation ( J:48412 )

• T cells primed with MBP proliferate in culture unlike wild-type cells

Allelic Composition	Mbp^shi/Mbp^shi
Genetic Background	involves: C3H * ICR * SWV

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Mbp^shi mutation (3 available); any Mbp mutation (32 available)

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

mortality/aging

premature death ( J:6578 )

• mice die between P50 and P100

• Background Sensitivity: mice on a mixed C3H, ICR, and SWV background live longer than mice on a pure SWV background

nervous system

environmentally induced seizures ( J:6578 )

• seizures can be triggered by sound, motion, light, and handling

audiogenic seizures ( J:6578 )

tonic seizures ( J:6578 )

• from P30

behavior/neurological

tremors ( J:6578 )

• beginning at 12 days when animals are active

hindlimb paralysis ( J:6578 )

• on rare occasion with age

environmentally induced seizures ( J:6578 )

• seizures can be triggered by sound, motion, light, and handling

audiogenic seizures ( J:6578 )

tonic seizures ( J:6578 )

• from P30

Allelic Composition	Mbp^shi/Mbp^shi
Genetic Background	involves: SWV

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Mbp^shi mutation (3 available); any Mbp mutation (32 available)

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

nervous system

abnormal neuron morphology ( J:57128 )

• neurons exhibit increased microtubule number and density and neurofilament density compared with wild-type cells

abnormal myelination ( J:57128 )

• mice lack compact myelin unlike wild-type mice

abnormal axonal transport ( J:57128 )

• increased

behavior/neurological

impaired coordination ( J:127688 )

• on a rotarod, mice exhibit impaired coordination compared with wild-type mice

• however, transgenic expression of Mbp or transplantation of wild-type oligodendrocytes improve performance on a rotarod

Allelic Composition	Mbp^shi/Mbp^shi
Genetic Background	SWV-Mbp^shi

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Mbp^shi mutation (3 available); any Mbp mutation (32 available)

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

mortality/aging

premature death ( J:6578 )

• mice die between P50 and P100

• Background Sensitivity: mice on a pure SWV background die sooner than mice on a mixed C3H, ICR, and SWV background

reproductive system

reproductive system phenotype ( J:6578 )

N	• mice are fertile

behavior/neurological

abnormal spatial learning ( J:27482 )

• in a T-maze, mice exhibit impaired successive reversal learning compared with wild-type mice

abnormal maternal nurturing ( J:6578 )

• clinical manifestations interfere with normal mothering

homeostasis/metabolism

decreased cholesterol level ( J:9907 )

• slightly in the sciatic nerve

decreased brain cholesterol level ( J:9907 )

nervous system

decreased brain cholesterol level ( J:9907 )

Allelic Composition	Mbp^shi/Mbp⁺
Genetic Background	C3Fe.SWV-Mbp^shi/J

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Mbp^shi mutation (3 available); any Mbp mutation (32 available)

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

hearing/vestibular/ear

absent linear vestibular evoked potential ( J:116914 )

• in one of two mice tested, VESPs are absent at the maximum stimulus intensity used

reduced linear vestibular evoked potential ( J:116914 )

• elevated threshold and absent central response peaks in aged mice

Allelic Composition	Mbp^shi/Mbp^shi Plp1^tm1Kan/Y
Genetic Background	involves: 129S1/Sv * 129X1/SvJ * C57BL/6J * SWV

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Mbp^shi mutation (3 available); any Mbp mutation (32 available)
Plp1^tm1Kan mutation (1 available); any Plp1 mutation (18 available)

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

mortality/aging

mortality/aging ( J:38856 )

N	• double mutants live for more than 6 months unlike Mbp^shi homozygotes that die around 3 months of age

nervous system

abnormal myelin sheath morphology ( J:38856 )

• when present axon sheaths are thinner than normal, often decompacted, lack major dense lines, and have intraperiod lines that are denser than normal

abnormal myelination ( J:38856 )

• dysmyelination with many naked axons

behavior/neurological

tremors ( J:38856 )

ataxia ( J:38856 )

Allelic Composition	Mbp^shi/Mbp^shi Plp1^tm1Kan/Y
Genetic Background	involves: 129S1/Sv * 129X1/SvJ * SWV

Find Mice

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

nervous system

axonal spheroids ( J:48031 )

• axonal spheroids are seen unlike in Mbp^shi homozygotes

demyelination ( J:48031 )

• severe hypomyelination

Allelic Composition	Mbp^shi/Mbp⁺ Mobp^tm1Irg/Mobp⁺
Genetic Background	involves: 129S1/SvImJ * C57BL/6J * SWV

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Mbp^shi mutation (3 available); any Mbp mutation (32 available)
Mobp^tm1Irg mutation (0 available); any Mobp mutation (30 available)

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

nervous system

nervous system phenotype ( J:104861 )

N	• normal with respect to axon diameter of the optic nerve

Allelic Composition	Mbp^shi/Mbp^shi Tg(Thy1-YFP)HJrs/0
Genetic Background	involves: C3HeB/Fe * C57BL/6 * CBA * SWV

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Mbp^shi mutation (3 available); any Mbp mutation (32 available)
Tg(Thy1-YFP)HJrs mutation (3 available)

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

nervous system

increased susceptibility to pharmacologically induced seizures ( J:158545 )

• following treatment with alpha-amino-3-hydroxy-5-methylisoxazole-4-propionic acid (AMPA), seizures are more frequent and last longer than in similarly treated wild-type mice

increased susceptibility to neuronal excitotoxicity ( J:158545 )

• mice treated with alpha-amino-3-hydroxy-5-methylisoxazole-4-propionic acid (AMPA) exhibit increased axonal damage, excitotoxicity, and behavioral deficits (including seizures, hindlimb and tail paresis, impaired coordination) compared with similarly treated wild-type mice

• however, activation of the N-methyl-D-aspartic acid (NMDA) receptors does not result in axonal injury

abnormal myelination ( J:158545 )

• dorsal columns lack myelin unlike in wild-type mice

• mice exhibit amyelinated and hypomyelinated axons unlike in wild-type mice

demyelination ( J:158545 )

• mice exhibit amyelinated and hypomyelinated axons unlike in wild-type mice

behavior/neurological

increased susceptibility to pharmacologically induced seizures ( J:158545 )

• following treatment with alpha-amino-3-hydroxy-5-methylisoxazole-4-propionic acid (AMPA), seizures are more frequent and last longer than in similarly treated wild-type mice

impaired coordination ( J:158545 )

• on a rotarod at baseline and after treatment with alpha-amino-3-hydroxy-5-methylisoxazole-4-propionic acid (AMPA)

paresis ( J:158545 )

• hindlimb and tail paresis following treatment with alpha-amino-3-hydroxy-5-methylisoxazole-4-propionic acid (AMPA)

homeostasis/metabolism

increased susceptibility to neuronal excitotoxicity ( J:158545 )

• however, activation of the N-methyl-D-aspartic acid (NMDA) receptors does not result in axonal injury

cellular

increased susceptibility to neuronal excitotoxicity ( J:158545 )

• however, activation of the N-methyl-D-aspartic acid (NMDA) receptors does not result in axonal injury

Allelic Composition	Mbp^shi/Mbp^shi Pmp2^{tm1.1(KOMP)Vlcg}/Pmp2^{tm1.1(KOMP)Vlcg}
Genetic Background	involves: C3HeB/FeJ * C57BL/6 * C57BL/6NTac

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Mbp^shi mutation (3 available); any Mbp mutation (32 available)
Pmp2^{tm1.1(KOMP)Vlcg} mutation (0 available); any Pmp2 mutation (13 available)

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

behavior/neurological

limb grasping ( J:211344 )

• mice show severe hind limb clasping at P56

tremors ( J:211344 )

• mice develop a severe generalized tremor during locomotion by 2 weeks of age, indicating CNS defects

nervous system

abnormal myelin sheath morphology ( J:211344 )

• mice show a significant increase in the number of Schmidt-Lanterman incisures on sciatic nerve cross sections relative to control mice

• however, peripheral nerve myelin morphology is not significantly altered at P10 and P56 and the distribution of Na_v- and K_v1.2-channels in teased sciatic nerve fibers is normal at P56, indicating that the structure of the node of Ranvier is preserved

decreased nerve conduction velocity ( J:211344 )

• at P56, mice show a significant reduction in motor nerve conduction velocity that is similar to that observed in Mbp^shi homozygotes

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