Phenotypes associated with this allele

• Allele Symbol: Tg(Mpz*S63X)31Mes
• Allele Name: transgene insertion 31, Albee Messing
• Allele ID: MGI:5824056
• Summary: 3 genotypes

Jump to	Allelic Composition	Genetic Background	Genotype ID
cx1	Mpz^tm1Msch/Mpz^+ Tg(Mpz*S63X)31Mes/0	involves: 129S7/SvEvBrd * FVB/N	MGI:6276577
cx2	Mpz^tm1Msch/Mpz^tm1Msch Tg(Mpz*S63X)31Mes/0	involves: 129S7/SvEvBrd * FVB/N	MGI:6276578
tg3	Tg(Mpz*S63X)31Mes/0	involves: FVB/N	MGI:6276576

Genotype
MGI:6276577

cx1

• Allelic Composition: Mpz^tm1Msch/Mpz⁺; Tg(Mpz*S63X)31Mes/0
• Genetic Background: involves: 129S7/SvEvBrd * FVB/N

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

behavior/neurological

impaired coordination ( J:105751 )

• mice are able to remain for only half as long on an accelerating rotarod compared to wild-type mice

nervous system

decreased myelin sheath thickness ( J:105751 )

• sciatic and digital nerves show thin myelin sheaths, more pronounced distally in digital nerves and progressing with age

axon degeneration ( J:105751 )

• occasional axonal degeneration in digital nerves

demyelination ( J:105751 )

• sciatic and digital nerves show onion bulbs and thin myelin sheaths, all more pronounced distally in digital nerves, and progressing with age

abnormal action potential ( J:105751 )

• compound muscle action potentials recorded from the small foot muscles after supramaximal proximal sciatic nerve stimulation show a trend toward reduced compound muscle action potential amplitude in both facial and sciatic nerves

• compound muscle action potentials show only minor temporal dispersion, indicating uniform changes throughout myelin

decreased nerve conduction velocity ( J:105751 )

• electrophysiological analysis in sciatic nerves shows slowed nerve conduction and prolonged F-wave latency

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
Charcot-Marie-Tooth disease type 1B		DOID:0110152	OMIM:118200	J:105751

Genotype
MGI:6276578

cx2

• Allelic Composition: Mpz^tm1Msch/Mpz^tm1Msch; Tg(Mpz*S63X)31Mes/0
• Genetic Background: involves: 129S7/SvEvBrd * FVB/N

nervous system

abnormal myelination ( J:105751 )

• myelin is uncompacted

Genotype
MGI:6276576

tg3

• Allelic Composition: Tg(Mpz*S63X)31Mes/0
• Genetic Background: involves: FVB/N

behavior/neurological

tremors ( J:105751 )

ataxia ( J:105751 )

weakness ( J:105751 )

muscle

muscular atrophy ( J:105751 )

• muscle atrophy in the hindlimbs

nervous system

abnormal nervous system physiology ( J:105751 )

• mice develop a neuromuscular disorder between 4-8 weeks of age

demyelination ( J:105751 )

• sciatic nerve shows demyelinating neuropathy

• onion bulbs, signifying myelin destruction followed by attempts to remyelinate, are present at 6 months of age and increase in number with age

• 6 month old mice show demyelination and occasional onion bulbs in both ventral (motor) and dorsal (sensory) roots as well as in sciatic nerve

dysmyelination ( J:105751 )

• hypomyelination with occasional nude axons

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
Charcot-Marie-Tooth disease type 1B		DOID:0110152	OMIM:118200	J:105751