Tg(Mpz)88.2Mfel
Transgene Detail

Summary

• Symbol: Tg(Mpz)88.2Mfel
• Name: transgene insertion 88.2, M Laura Feltri
• MGI ID: MGI:2384593
• Synonyms: 88.2,TgN(Mpzmyc)20Mes, P0myc/Tg88.2
• Transgene: Tg(Mpz)88.2Mfel Location: unknown
• Alliance: Tg(Mpz)88.2Mfel page

Transgene origin

• Strain of Origin: FVB/N

Transgene description

• Transgene Type: Transgenic (Inserted expressed sequence)
• Mutation: Insertion
• Tg(Mpz)88.2Mfel expresses 1 gene
  Transgene expresses:
  

  Organism	Expressed Gene	Note
  mouse	Mpz (MGI:103177)

• Mutation details: The entire Mpz gene (including 6 kb of promoter sequences, all exons and introns, and the natural polyadenylation site) with an in-frame myc tag insertion in exon 2 was used as the transgene. The myc tag is inserted downstream of the signal peptide sequence and precedes the extracellular domain of the mature protein at the N-terminus. (J:77658)

Disease models

Expression

In Structures Affected by this Mutation:

1 anatomical structure(s)

Find Mice (IMSR)

• Mouse strains and cell lines available from the International Mouse Strain Resource (IMSR)
• Carrying this Mutation: Mouse Strains: 0 strains available Cell Lines: 0 lines available

Notes

Founder 88.2 exhibits muscle atrophy, tremors, and muscle weakness (paw clenching, dragging of the hind limbs, floppy tail) with the most severe phenotype out of 3 different transgenic lines. Overexpression of the transgene in founder 88.2 is approximately 4-fold that of the endogenous protein. Morphology of the sciatic nerve in transgenic animals resembles that of Charcot-Marie-Tooth subtype 1B (CMTB1) patients with mutations in the Mpz gene.

References

• Original: J:77658 Previtali SC, et al., Epitope-tagged P(0) glycoprotein causes Charcot-Marie-Tooth-like neuropathy in transgenic mice. J Cell Biol. 2000 Nov 27;151(5):1035-46
• All: 1 reference(s)