FVB-Tg(Mpz*L106I)3Msch/Cnrm
| Status | Available to order |
| EMMA ID | EM:00314 |
| Citation information | RRID:IMSR_EM:00314 Research Resource Identifiers (RRID) are persistent unique ID numbers assigned to help researchers cite key resources (e.g. antibodies, model organisms and software projects) in the biomedical literature to improve transparency and reproducibility in research. See https://www.rrids.org/ for more information. |
| 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