FVB-Tg(Mpz*Ala221fs)4Msch/Cnrm
| Status | Available to order |
| EMMA ID | EM:00315 |
| International strain name | FVB-Tg(Mpz*Ala221fs)4Msch/Cnrm |
| Alternative name | P0ins4 |
| Strain type | Transgenic Strains |
| Allele/Transgene symbol | Tg(Mpz*Ala221fs)4Msch, |
| Gene/Transgene symbol | Tg(Mpz*Ala221fs)4Msch |
Information from provider
| Provider | Melitta Schachner |
| Provider affiliation | Center for molecular neurobiology Hamburg, Center for molecular neurobiology Hamburg |
| Genetic information | To generate a mouse line that expresses the pathogenic insertion mutation c.662_663GC (P0ins, Ekici et al., 2002, Rautenstrauss et al., 1994, Fig.1b), a 97bp human MPZ (P0) cDNA fragment, starting at the second nucleotide of exon 6 and comprising the GC insertion, was exchanged against the corresponding sequence in the mouse Mpz gene using the Seamless genotyping cloning Kit. In brief, the human DNA sequence was amplified from the pRmha3-P0 vector, containing a 0.9kb-EcoRI-SacI-fragment of the human P0cDNA including the GC insertion mutation. This fragment was ligated into the mP0NA vector (Lehmke et al., 1988, You et al., 1991) that includes a 1kb-NcoI-AatII fragment with exon 5 to 6 of the mouse Mpz gene and which has been completely amplified with the exception of the sequence that corresponds to the human fragment. A 1kb-NcoI-AatII-fragment of the resulting mP0NA-P0ins vector was subcloned into the NcoI/AatII- digested mP05.7blue vector. |
| Phenotypic information | Thin, compact myelin sheaths, mild gait abnormalities. Model of Dejerine-Sottas syndrome (DSS). |
| 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