Primary Identifier | MGI:2384592 | Allele Type | Transgenic |
Attribute String | Inserted expressed sequence | Gene | Tg(Mpz)88.1Mfel |
Strain of Origin | FVB/N | Is Recombinase | false |
Is Wild Type | false |
description | 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. |
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. |