| Primary Identifier | MGI:2384593 | Allele Type | Transgenic |
| Attribute String | Inserted expressed sequence | Gene | Tg(Mpz)88.2Mfel |
| Strain of Origin | FVB/N | Is Recombinase | false |
| Is Wild Type | false |
| description | 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. |
| molecularNote | 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. |
