Tg(Mpz)88.1Mfel
Transgene Detail

Summary

• Symbol: Tg(Mpz)88.1Mfel
• Name: transgene insertion 88.1, M Laura Feltri
• MGI ID: MGI:2384592
• Synonyms: 88.1,TgN(Mpzmyc)19Mes, P0myc/Tg88.1
• Transgene: Tg(Mpz)88.1Mfel Location: unknown
• Alliance: Tg(Mpz)88.1Mfel page

Transgene origin

• Strain of Origin: FVB/N

Transgene description

• Transgene Type: Transgenic (Inserted expressed sequence)
• Mutation: Insertion
• Tg(Mpz)88.1Mfel 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 88.1 exhibits muscle atrophy, tremors, and muscle weakness (paw clenching, dragging of the hind limbs, floppy tail) with intermediate severity. Overexpression of the transgene in founder 88.1 is approximately 2.5-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)