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Truncated CBP protein leads to classical Rubinstein-Taybi syndrome phenotypes in mice: implications for a dominant-negative mechanism.

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.

Pubmed ID: 9949198


  • Oike Y
  • Hata A
  • Mamiya T
  • Kaname T
  • Noda Y
  • Suzuki M
  • Yasue H
  • Nabeshima T
  • Araki K
  • Yamamura K


Human molecular genetics

Publication Data

March 3, 1999

Associated Grants


Mesh Terms

  • Animals
  • Base Sequence
  • Behavior, Animal
  • CREB-Binding Protein
  • DNA Primers
  • Disease Models, Animal
  • Female
  • Genes, Dominant
  • Heterozygote
  • Humans
  • Male
  • Memory
  • Mice
  • Mice, Mutant Strains
  • Motor Activity
  • Mutagenesis, Insertional
  • Nuclear Proteins
  • Phenotype
  • Pregnancy
  • Rubinstein-Taybi Syndrome
  • Sequence Deletion
  • Trans-Activators