All Phenotypes Gpm6b<tm1Kan> MGI Mouse

Phenotypes associated with this allele

Jump to	Allelic Composition	Genetic Background	Genotype ID
hm1	Gpm6b^tm1Kan/Gpm6b^tm1Kan	involves: 129S1/Sv * 129X1/SvJ * C57BL/6J	MGI:5487807
cx2	Gpm6b^tm1Kan Plp1^tm1Kan/Y	involves: 129S1/Sv * 129X1/SvJ * C57BL/6J	MGI:5487810
cx3	Gpm6b^tm1Kan/Gpm6b^tm1Kan Plp1^tm1Kan/Plp1⁺	involves: 129S1/Sv * 129X1/SvJ * C57BL/6J	MGI:5487811
cx4	Gpm6b^tm1Kan/Gpm6b⁺ Plp1^tm1Kan/Plp1^tm1Kan	involves: 129S1/Sv * 129X1/SvJ * C57BL/6J	MGI:5487812
ot5	Gpm6b^tm1Kan/Y	involves: 129S1/Sv * 129X1/SvJ * C57BL/6J	MGI:5487808

Allelic Composition	Gpm6b^tm1Kan/Gpm6b^tm1Kan
Genetic Background	involves: 129S1/Sv * 129X1/SvJ * C57BL/6J

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Gpm6b^tm1Kan mutation (0 available); any Gpm6b mutation (6 available)

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

nervous system

abnormal axon morphology ( J:193310 )

• irregularities are detected at the axon-myelin interface including; irregular width of the extracellular periaxonal space and occasional apparent fusion of the adaxonal glial membrane with the axonal membrane

• occasionally the axon-myelin apposition is severely disturbed

• rarely invaginations of the oligodendroglial plasma membrane into the axoplasm are seen

abnormal myelin sheath morphology ( J:193310 )

• double myelination is occasionally seen

behavior/neurological

behavior/neurological phenotype ( J:193310 )

N	• no defects in motor function are detected in mice at up to 24 months of age

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

mortality/aging

premature death ( J:193310 )

• most die by 4 - 5 months of age with none living past 6 months of age

behavior/neurological

tremors ( J:193310 )

abnormal motor coordination/balance ( J:193310 )

• unable to remain on a horizontal bar without support at all ages tested

ataxia ( J:193310 )

hunched posture ( J:193310 )

• hunchback at 2 - 3 months of age

abnormal stationary movement ( J:193310 )

• fail to grasp objects with the forelimbs

muscle

muscle hypertonia ( J:193310 )

• hindlimb spasticity is seen at 10 days of age

muscle spasm ( J:193310 )

• at 2 - 3 months of age mice always have spastic hindlimbs

nervous system

abnormal oligodendrocyte apoptosis ( J:193310 )

• apoptotic oligodendrocytes are seen in the CNS

enlarged brain ventricles ( J:193310 )

abnormal brain white matter morphology ( J:193310 )

• pathology is more severe in the white matter than in the gray matter

decreased corpus callosum size ( J:193310 )

• thickness is reduced to 2/3 that of controls

decreased anterior commissure size ( J:193310 )

• thickness is reduced to 2/3 that of controls

abnormal glial cell morphology ( J:193310 )

microgliosis ( J:193310 )

• reactive microgliosis accompanies neurodegeneration

astrocytosis ( J:193310 )

• accompanies neurodegeneration

abnormal oligodendrocyte morphology ( J:193310 )

• processes are longer and thicker and are often seen engulfing axons with a noncompacted cellular process

abnormal myelin sheath morphology ( J:193310 )

• absence of proteolipids, decrease in cholesterol content

• intraperiod lines are partly condensed

neuron degeneration ( J:193310 )

• pathology is more severe in the white matter than in the gray matter

• degeneration is also seen in the retina and the PNS

axon degeneration ( J:193310 )

• axonal swellings are seen earlier and more frequently than in Plp single null mice

• axonal degeneration in the optic nerve at 4.5 months of age

abnormal myelination ( J:193310 )

• at P14 the optic nerve lacks myelin and at 1 month of age myelination of the optic nerve and spinal cord are clearly delayed

• myelination in the striatal white matter and in spinal cord gray matter is 86% and 67% that of controls

• however, most large caliber axons are myelinated

demyelination ( J:193310 )

vision/eye

retina degeneration ( J:193310 )

absent visual evoked potential ( J:193310 )

• no reproducible responses are detected at 19 - 25 weeks of age

hearing/vestibular/ear

hearing/vestibular/ear phenotype ( J:193310 )

N	• distortion product otoacoustic emissions are present and major degeneration of the VIIIth cranial nerve is not seen

abnormal auditory brainstem response waveform shape ( J:193310 )

• waves II - V (reflecting CNS function) are significantly delayed in mice at 2.5 months of age

• the delay in waves II - V is less pronounced at 4.5 months of age

• at both 2.5 and 4.5 months of age wave III is most delayed

impaired hearing ( J:193310 )

• moderate pantonal hearing loss across all frequencies

cellular

abnormal oligodendrocyte apoptosis ( J:193310 )

• apoptotic oligodendrocytes are seen in the CNS

increased apoptosis ( J:193310 )

• apoptotic cells are seen in the CNS, some of which are oligodendrocytes

hematopoietic system

microgliosis ( J:193310 )

• reactive microgliosis accompanies neurodegeneration

immune system

microgliosis ( J:193310 )

• reactive microgliosis accompanies neurodegeneration

homeostasis/metabolism

decreased cholesterol level ( J:193310 )

• in myelin

Allelic Composition	Gpm6b^tm1Kan/Gpm6b^tm1Kan Plp1^tm1Kan/Plp1⁺
Genetic Background	involves: 129S1/Sv * 129X1/SvJ * C57BL/6J

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Gpm6b^tm1Kan mutation (0 available); any Gpm6b mutation (6 available)
Plp1^tm1Kan mutation (1 available); any Plp1 mutation (18 available)

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

mortality/aging

premature death ( J:193310 )

• die at 7 - 12 months of age later than males

behavior/neurological

abnormal motor capabilities/coordination/movement ( J:193310 )

• progressive motor impairments similar to males but milder at 4 - 6 weeks of age

Allelic Composition	Gpm6b^tm1Kan/Gpm6b⁺ Plp1^tm1Kan/Plp1^tm1Kan
Genetic Background	involves: 129S1/Sv * 129X1/SvJ * C57BL/6J

Find Mice

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

mortality/aging

premature death ( J:193310 )

• die at 7 - 12 months of age later than males

behavior/neurological

abnormal motor capabilities/coordination/movement ( J:193310 )

• progressive motor impairments similar to males but milder at 4 - 6 weeks of age

Allelic Composition	Gpm6b^tm1Kan/Y
Genetic Background	involves: 129S1/Sv * 129X1/SvJ * C57BL/6J

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Gpm6b^tm1Kan mutation (0 available); any Gpm6b mutation (6 available)

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

nervous system

abnormal axon morphology ( J:193310 )

• occasionally the axon?myelin apposition is severely disturbed

• rarely invaginations of the oligodendroglial plasma membrane into the axoplasm are seen

abnormal myelin sheath morphology ( J:193310 )

• double myelination is occasionally seen

behavior/neurological

behavior/neurological phenotype ( J:193310 )

N	• no defects in motor function are detected in mice at up to 24 months of age

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