| First Author | Shmerling D | Year | 1998 |
| Journal | Cell | Volume | 93 |
| Issue | 2 | Pages | 203-14 |
| PubMed ID | 9568713 | Mgi Jnum | J:47034 |
| Mgi Id | MGI:1202515 | Doi | 10.1016/s0092-8674(00)81572-x |
| Citation | Shmerling D, et al. (1998) Expression of amino-terminally truncated PrP in the mouse leading to ataxia and specific cerebellar lesions. Cell 93(2):203-14 |
| abstractText | The physiological role of prion protein (PrP) remains unknown. Mice devoid of PrP develop normally but are resistant to scrapie; introduction of a PrP transgene restores susceptibility to the disease. To identify the regions of PrP necessary for this activity, we prepared PrP knockout mice expressing PrPs with amino-proximal deletions. Surprisingly, PrP lacking residues 32-121 or 32-134, but not with shorter deletions, caused severe ataxia and neuronal death limited to the granular layer of the cerebellum as early as 1-3 months after birth. The defect was completely abolished by introducing one copy of a wild-type PrP gene. We speculate that these truncated PrPs may be nonfunctional and compete with some other molecule with a PrP-like function for a common ligand. |
