| First Author | Oike Y | Year | 1999 |
| Journal | Hum Mol Genet | Volume | 8 |
| Issue | 3 | Pages | 387-96 |
| PubMed ID | 9949198 | Mgi Jnum | J:53370 |
| Mgi Id | MGI:1332352 | Doi | 10.1093/hmg/8.3.387 |
| Citation | Oike Y, et al. (1999) Truncated CBP protein leads to classical Rubinstein-Taybi syndrome phenotypes in mice: implications for a dominant-negative mechanism. Hum Mol Genet 8(3):387-96 |
| abstractText | A mouse model of Rubinstein-Taybi syndrome (RTS) was generated by an insertional mutation into the cyclic AMP response element-binding protein (CREB)-binding protein (CBP) gene. Heterozygous CBP-deficient mice, which had truncated CBP protein (residues 1-1084) containing the CREB-binding domain (residues 462-661), showed clinical features of RTS, such as growth retardation (100%), retarded osseous maturation (100%), hypoplastic maxilla with narrow palate (100%), cardiac anomalies (15%) and skeletal abnormalities (7%), Truncated CBP is considered to have been acting during development as a dominant- negative inhibitor to lead to the phenotypes of RTS in mice, Our studies with step-through-type passive avoidance tests and with fear conditioning test showed that mice were deficient in long-term memory (LTM), In contrast, short-term memory (STM) appeared to be normal. These results implicate a crucial role for CBP in mammalian LTM, Our CBP+/- mice would be an excellent model for the study of the role of CBP in development and memory storage mechanisms. |
