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Phenotypes associated with this allele
Allele Symbol
Allele Name
Allele ID 		Plp1tm1Kan
targeted mutation 1, Klaus-Armin Nave
MGI:1857446
Summary 13 genotypes
Jump to Allelic Composition Genetic Background Genotype ID
ht1
 		Plp1tm1Kan/Plp1+ involves: 129S1/Sv * 129X1/SvJ MGI:3620248
cx2
 		Plp1tm1Kan/Y
Tspan2tm1Hbw/Tspan2tm1Hbw 		B6.Cg-Tspan2tm1Hbw Plp1tm1Kan MGI:5556059
cx3
 		Plp1tm1Kan/Y
Cnptm1(cre)Kan/Cnp+ 		involves: 129S1/Sv * 129X1/SvJ MGI:6160760
cx4
 		Gpm6btm1KanPlp1tm1Kan/Y involves: 129S1/Sv * 129X1/SvJ * C57BL/6J MGI:5487810
cx5
 		Gpm6btm1Kan/Gpm6btm1Kan
Plp1tm1Kan/Plp1+ 		involves: 129S1/Sv * 129X1/SvJ * C57BL/6J MGI:5487811
cx6
 		Gpm6btm1Kan/Gpm6b+
Plp1tm1Kan/Plp1tm1Kan 		involves: 129S1/Sv * 129X1/SvJ * C57BL/6J MGI:5487812
cx7
 		Mbpshi/Mbpshi
Plp1tm1Kan/Y 		involves: 129S1/Sv * 129X1/SvJ * C57BL/6J * SWV MGI:3620243
cx8
 		Mbpshi/Mbpshi
Plp1tm1Kan/Y 		involves: 129S1/Sv * 129X1/SvJ * SWV MGI:3620245
ot9
 		Plp1tm1Kan/Y B6.129-Plp1tm1Kan MGI:5556060
ot10
 		Plp1tm1Kan/Y B6N.129-Plp1tm1Kan MGI:5902381
ot11
 		Plp1tm1Kan/Y involves: 129S1/Sv * 129X1/SvJ MGI:3620242
ot12
 		Plp1tm1Kan/Y involves: 129S1/Sv * 129X1/SvJ * C57BL/6J MGI:3620241
ot13
 		Plp1tm1Kan/? involves: 129S1/Sv * 129X1/SvJ * C57BL/6 MGI:3838180


Genotype
MGI:3620248
ht1
Allelic
Composition		 Plp1tm1Kan/Plp1+
Genetic
Background		 involves: 129S1/Sv * 129X1/SvJ
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Plp1tm1Kan mutation (1 available); any Plp1 mutation (18 available)
♀ phenotype observed in females
♂ phenotype observed in males
N normal phenotype
nervous system
abnormal axon morphology ( J:48031 )
• axonal swelling and degeneration are seen but less frequently than in hemizygous males; however all optic nerve axons have normal myelin sheaths




Genotype
MGI:5556059
cx2
Allelic
Composition		 Plp1tm1Kan/Y
Tspan2tm1Hbw/Tspan2tm1Hbw
Genetic
Background		 B6.Cg-Tspan2tm1Hbw Plp1tm1Kan
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Plp1tm1Kan mutation (1 available); any Plp1 mutation (18 available)
Tspan2tm1Hbw mutation (0 available); any Tspan2 mutation (11 available)
♀ phenotype observed in females
♂ phenotype observed in males
N normal phenotype
nervous system
microgliosis ( J:201143 )
• increased density of microglia in the hippocampal fimbria
• microglial cells appear to be phagocytic based on presence of inflated lysosomes
brain inflammation ( J:201143 )
• infiltration of cytotoxic T cells and macrophages in the hippocampal fimbria
astrocytosis ( J:201143 )
• severe reactive astrogliosis in the hippocampal fimbria
• enhanced compared to single mutants
axon degeneration ( J:201143 )
• similar to what is seen in mice hemizygous for Plp1tm1Kan alone
axonal spheroids ( J:201143 )
• similar to what is seen in mice hemizygous for Plp1tm1Kan alone
demyelination ( J:201143 )
• moderately dysmyelinated, similar to what is seen in mice hemizygous for Plp1tm1Kan alone

behavior/neurological
hindlimb paralysis ( J:201143 )
• at 40 weeks of age

growth/size/body
decreased body weight ( J:201143 )
• at 4 and 40 weeks of age
• similar to what is seen in mice hemizygous for Plp1tm1Kan alone

skeleton
kyphosis ( J:201143 )
• at 40 weeks of age

immune system
microgliosis ( J:201143 )
• increased density of microglia in the hippocampal fimbria
• microglial cells appear to be phagocytic based on presence of inflated lysosomes
brain inflammation ( J:201143 )
• infiltration of cytotoxic T cells and macrophages in the hippocampal fimbria

hematopoietic system
microgliosis ( J:201143 )
• increased density of microglia in the hippocampal fimbria
• microglial cells appear to be phagocytic based on presence of inflated lysosomes




Genotype
MGI:6160760
cx3
Allelic
Composition		 Plp1tm1Kan/Y
Cnptm1(cre)Kan/Cnp+
Genetic
Background		 involves: 129S1/Sv * 129X1/SvJ
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Cnptm1(cre)Kan mutation (0 available); any Cnp mutation (27 available)
Plp1tm1Kan mutation (1 available); any Plp1 mutation (18 available)
♀ phenotype observed in females
♂ phenotype observed in males
N normal phenotype
hematopoietic system
increased T cell number ( J:245100 )
• density of T-lymphocytes (CD3+ cells) in the fimbria is further increased at 26 weeks of age compared to single hemizygous Plp1 males
microgliosis ( J:245100 )
• microgliosis in the hippocampal fimbria is further increased at 26 weeks of age compared to single hemizygous Plp1 males

immune system
increased T cell number ( J:245100 )
• density of T-lymphocytes (CD3+ cells) in the fimbria is further increased at 26 weeks of age compared to single hemizygous Plp1 males
microgliosis ( J:245100 )
• microgliosis in the hippocampal fimbria is further increased at 26 weeks of age compared to single hemizygous Plp1 males

nervous system
microgliosis ( J:245100 )
• microgliosis in the hippocampal fimbria is further increased at 26 weeks of age compared to single hemizygous Plp1 males
abnormal hippocampal fimbria morphology ( J:245100 )
• hippocampal fimbria exhibits APP+ axonal spheroids, microgliosis and astrogliosis, and increased density of T-lymphocytes
astrocytosis ( J:245100 )
• moderate, but significant, astrogliosis in the hippocampal fimbria at 26 weeks of age
axonal spheroids ( J:245100 )
• APP+ axonal spheroids are present in the hippocampal fimbria and corpus callosum at 26 weeks of age at enhanced levels compared to single hemizygous Plp1 males




Genotype
MGI:5487810
cx4
Allelic
Composition		 Gpm6btm1KanPlp1tm1Kan/Y
Genetic
Background		 involves: 129S1/Sv * 129X1/SvJ * C57BL/6J
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Gpm6btm1Kan mutation (0 available); any Gpm6b mutation (6 available)
Plp1tm1Kan 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 )
• most die by 4 - 5 months of age with none living past 6 months of age

behavior/neurological
abnormal motor coordination/balance ( J:193310 )
• unable to remain on a horizontal bar without support at all ages tested
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
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
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

vision/eye
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




Genotype
MGI:5487811
cx5
Allelic
Composition		 Gpm6btm1Kan/Gpm6btm1Kan
Plp1tm1Kan/Plp1+
Genetic
Background		 involves: 129S1/Sv * 129X1/SvJ * C57BL/6J
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Gpm6btm1Kan mutation (0 available); any Gpm6b mutation (6 available)
Plp1tm1Kan 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




Genotype
MGI:5487812
cx6
Allelic
Composition		 Gpm6btm1Kan/Gpm6b+
Plp1tm1Kan/Plp1tm1Kan
Genetic
Background		 involves: 129S1/Sv * 129X1/SvJ * C57BL/6J
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Gpm6btm1Kan mutation (0 available); any Gpm6b mutation (6 available)
Plp1tm1Kan 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




Genotype
MGI:3620243
cx7
Allelic
Composition		 Mbpshi/Mbpshi
Plp1tm1Kan/Y
Genetic
Background		 involves: 129S1/Sv * 129X1/SvJ * C57BL/6J * SWV
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Mbpshi mutation (3 available); any Mbp mutation (32 available)
Plp1tm1Kan 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 Mbpshi 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




Genotype
MGI:3620245
cx8
Allelic
Composition		 Mbpshi/Mbpshi
Plp1tm1Kan/Y
Genetic
Background		 involves: 129S1/Sv * 129X1/SvJ * SWV
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Mbpshi mutation (3 available); any Mbp mutation (32 available)
Plp1tm1Kan mutation (1 available); any Plp1 mutation (18 available)
♀ phenotype observed in females
♂ phenotype observed in males
N normal phenotype
nervous system
axonal spheroids ( J:48031 )
• axonal spheroids are seen unlike in Mbpshi homozygotes
demyelination ( J:48031 )
• severe hypomyelination




Genotype
MGI:5556060
ot9
Allelic
Composition		 Plp1tm1Kan/Y
Genetic
Background		 B6.129-Plp1tm1Kan
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Plp1tm1Kan mutation (1 available); any Plp1 mutation (18 available)
♀ phenotype observed in females
♂ phenotype observed in males
N normal phenotype
nervous system
microgliosis ( J:201143 )
• low grade in the hippocampal fimbria
astrocytosis ( J:201143 )
• mild astrogliosis in the hippocampal fimbria
axon degeneration ( J:201143 )
• density of axons shows a striking reduction to fewer than half that of controls
demyelination ( J:201143 )
• moderately dysmyelinated due to diminished myelination of axons with the smallest calibers, significant at 40 weeks of age

behavior/neurological
hindlimb paralysis ( J:201143 )
• at 40 weeks of age

growth/size/body
decreased body weight ( J:201143 )
• at 4 and 40 weeks of age

skeleton
kyphosis ( J:201143 )
• at 40 weeks of age

immune system
microgliosis ( J:201143 )
• low grade in the hippocampal fimbria

hematopoietic system
microgliosis ( J:201143 )
• low grade in the hippocampal fimbria




Genotype
MGI:5902381
ot10
Allelic
Composition		 Plp1tm1Kan/Y
Genetic
Background		 B6N.129-Plp1tm1Kan
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Plp1tm1Kan mutation (1 available); any Plp1 mutation (18 available)
♀ phenotype observed in females
♂ phenotype observed in males
N normal phenotype
nervous system
dysmyelination ( J:236408 )
• hypomyelination with split myelin lamellae




Genotype
MGI:3620242
ot11
Allelic
Composition		 Plp1tm1Kan/Y
Genetic
Background		 involves: 129S1/Sv * 129X1/SvJ
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Plp1tm1Kan mutation (1 available); any Plp1 mutation (18 available)
♀ phenotype observed in females
♂ phenotype observed in males
N normal phenotype
nervous system
abnormal microglial cell morphology ( J:48031 )
• increased numbers of microglia accompany degenerative changes
microgliosis ( J:245100 )
• microgliosis in the hippocampal fimbria at 26 weeks of age
abnormal hippocampal fimbria morphology ( J:245100 )
• hippocampal fimbria exhibits APP+ axonal spheroids, microgliosis and astrogliosis
astrocytosis ( J:48031 , J:245100 )
• mild astrocytosis accompanies degenerative changes (J:48031)
• moderate, but significant, astrogliosis in the hippocampal fimbria at 26 weeks of age (J:245100)
abnormal oligodendrocyte morphology ( J:48031 , J:106182 )
• occasionally inner tongue processes of pligdendrocytes contain degenerated organelles (J:48031)
• primary glial cultures have increased numbers of oligodendrocytes starting at the second division and persisting through at least the fourteenth division; however no increase in oligodendrocyte proliferation is seen (J:106182)
• in primary glial cultures oligodendrocyte sheets appear larger, vacuolated, and broken suggesting decreased stability of the myelin sheet (J:106182)
abnormal axon morphology ( J:48031 )
• at 6 - 8 weeks, focal axonal swellings containing organelles are seen mostly in areas where small diameter axons predominate throughout the white and gray matter
• by 1 year, numerous large axonal swellings are seen in the optic nerve and spinal cord with some also seen in Purkinje cell axons
• after 1 year, axonal degeneration is seen in the optic nerve and fasciculus gracilis; however, no signs of peripheral neuropathy are seen
abnormal myelin sheath morphology ( J:48031 , J:193310 )
• in areas of axonal swelling the myelin sheath becomes attenuated and is eventually lost though slippage (J:48031)
• decrease in cholesterol content (J:193310)
axonal spheroids ( J:245100 )
• APP+ axonal spheroids are present in the hippocampal fimbria and corpus callosum at 26 weeks of age
demyelination ( J:48031 )
• evidence of demyelination is seen at 22 months of age

behavior/neurological
impaired coordination ( J:48031 )
• at 16 months of age impaired performance in a rotarod test is seen
abnormal locomotor coordination ( J:48031 )
• at 16 months of age impaired performance in a rotarod test is seen
abnormal gait ( J:48031 )
• older mice develop a slow gait

hematopoietic system
abnormal microglial cell morphology ( J:48031 )
• increased numbers of microglia accompany degenerative changes
microgliosis ( J:245100 )
• microgliosis in the hippocampal fimbria at 26 weeks of age

immune system
abnormal microglial cell morphology ( J:48031 )
• increased numbers of microglia accompany degenerative changes
microgliosis ( J:245100 )
• microgliosis in the hippocampal fimbria at 26 weeks of age

homeostasis/metabolism

Mouse Models of Human Disease
 DO ID OMIM ID(s) Ref(s)
hereditary spastic paraplegia 2 DOID:0110773 OMIM:312920
 J:48031 , J:245100




Genotype
MGI:3620241
ot12
Allelic
Composition		 Plp1tm1Kan/Y
Genetic
Background		 involves: 129S1/Sv * 129X1/SvJ * C57BL/6J
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Plp1tm1Kan mutation (1 available); any Plp1 mutation (18 available)
♀ phenotype observed in females
♂ phenotype observed in males
N normal phenotype
nervous system
abnormal myelin sheath morphology ( J:38856 )
• myelin is compacted but the distinction between major dense lines and intraperiod lines is less than in wild-type; however, the proportion of myelinated axons, myelin sheath thickness and size range of myelinated fibers are similar to wild-type
• in a few areas double intraperiod lines are seen and myelin sheaths are more susceptible to damage during the fixation process compared to wild-type
axonal spheroids ( J:38856 )
• in mice over 2 months of age, some axonal spheroids

behavior/neurological
behavior/neurological phenotype ( J:38856 )
N
• no signs of motor dysfunction or impaired coordination are detected




Genotype
MGI:3838180
ot13
Allelic
Composition		 Plp1tm1Kan/?
Genetic
Background		 involves: 129S1/Sv * 129X1/SvJ * C57BL/6
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Plp1tm1Kan mutation (1 available); any Plp1 mutation (18 available)
♀ phenotype observed in females
♂ phenotype observed in males
N normal phenotype
nervous system
abnormal axon morphology ( J:146665 )
• swollen axons are seen
abnormal myelin sheath morphology ( J:146665 )
• some attenuated myelin sheaths are seen around swollen axons
• however, in non swollen regions sheaths are of normal thickness
axon degeneration ( J:146665 )
• at 8 months of age occasional degenerating fibers are seen in the cervical region of the fasciculus gracilis
• at 18 months of age pronounced axonal degeneration is seen in the cervical segments in the thoracic region but not in the lumbar dorsal columns

behavior/neurological
ataxia ( J:146665 )
• gait ataxia is seen by 18 months of age
paraparesis ( J:146665 )
• weakness particularly of the hind limbs develops by 18 months of age

Mouse Models of Human Disease
 DO ID OMIM ID(s) Ref(s)
Pelizaeus-Merzbacher disease DOID:3210 OMIM:312080
 J:146665




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11/12/2024
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