FVB-Tg(Mpz*L106I)3Msch/Cnrm
| Status | Available to order |
| EMMA ID | EM:00314 |
| International strain name | FVB-Tg(Mpz*L106I)3Msch/Cnrm |
| Alternative name | P0sub3 |
| Strain type | Transgenic Strains |
| Allele/Transgene symbol | Tg(Mpz*L106I)3Msch |
| Gene/Transgene symbol | Tg(Mpz*L106I)3Msch |
Information from provider
| Provider | Melitta Schachner |
| Provider affiliation | Center for molecular neurobiology Hamburg, Center for molecular neurobiology Hamburg |
| Genetic information | To generate a mouse mutant expressing the pathogenic substitution Mpz (P0) I106L (P0sub, Gabreels-Festen et al., 1996, Fig. 1a) under the control of the Mpz promoter (Feltri et al., 1999), the 2.6BsrGI-sense vector, containing a 2.6kb-BsrGI fragment with exons 2 to 5 of the mouse P0 genomic DNA, was used for mutagenesis. In brief, the whole vector was amplified with primers flanking and containing the mutation and was religated. A StuI/Eco47II-fragment encoding exon 2 and mutated exon 3 of the resulting 2.6BsrGI-sense P0sub vector was cloned into the mP05.7blue vector (Lemke et al., 1988, Feltri et al., 1999), containing exons 2 to 6. Finally, an EcoRI fragment of the vector mP0Ewtblue (You et al.,1991, Feltri et al.,1999) comprising the Mpz promoter and exon 1 was inserted into the EcoRI digested mP05.7. |
| Phenotypic information | Distal muscle weakness and atrophy, reduced nerve conduction velocities and hypo- and dysmyelination (aberrantly folded myelin sheaths, tomacula) with "onion bulb" formation. In contrast to the P0sub1 strain (EMMA strain EM:00313), this strain contains Mpz (P0) amounts in the myelin fraction which are comparable to wild-type levels. Model of type 1b Charcot-Marie-Tooth disease. |
| Breeding history | The strain was always bred on a FVB background. |
| References |
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Information from EMMA
| Archiving centre | CNR, Consiglio Nazionale delle Ricerche, Monterotondo, Italy |
Literature references
- Pathology of a mouse mutation in peripheral myelin protein P0 is characteristic of a severe and early onset form of human Charcot-Marie-Tooth type 1B disorder.;Rünker Annette E, Kobsar Igor, Fink Torsten, Loers Gabriele, Tilling Thomas, Putthoff Peggy, Wessig Carsten, Martini Rudolf, Schachner Melitta, ;2004;The Journal of cell biology;165;565-73; 15148307