All Phenotypes Tg(Mpz*S63X)31Mes MGI Mouse

Phenotypes associated with this allele

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

♀	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

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

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Mpz^tm1Msch mutation (0 available); any Mpz mutation (28 available)
Tg(Mpz*S63X)31Mes mutation (0 available)

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

nervous system

abnormal myelination ( J:105751 )

• myelin is uncompacted

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

Find Mice

Using the International Mouse Strain Resource (IMSR)
No mouse lines available in IMSR.
See publication links below for author information.

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

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

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