A CBP binding transcriptional repressor produced by the PS1/epsilon-cleavage of N-cadherin is inhibited by PS1 FAD mutations.

Presenilin1 (PS1), a protein implicated in Alzheimer's disease (AD), forms complexes with N-cadherin, a transmembrane protein with important neuronal and synaptic functions. Here, we show that a PS1-dependent gamma-secretase protease activity promotes an epsilon-like cleavage of N-cadherin to produce its intracellular domain peptide, N-Cad/CTF2. NMDA receptor agonists stimulate N-Cad/CTF2 production ...
suggesting that this receptor regulates the epsilon-cleavage of N-cadherin. N-Cad/CTF2 binds the transcription factor CBP and promotes its proteasomal degradation, inhibiting CRE-dependent transactivation. Thus, the PS1-dependent epsilon-cleavage product N-Cad/CTF2 functions as a potent repressor of CBP/CREB-mediated transcription. Importantly, PS1 mutations associated with familial AD (FAD) and a gamma-secretase dominant-negative mutation inhibit N-Cad/CTF2 production and upregulate CREB-mediated transcription indicating that FAD mutations cause a gain of transcriptional function by inhibiting production of transcriptional repressor N-Cad/CTF2. These data raise the possibility that FAD mutation-induced transcriptional abnormalities maybe causally related to the dementia associated with FAD.
Mesh Terms:
Amyloid Precursor Protein Secretases, Animals, Aspartic Acid Endopeptidases, Blotting, Western, Cadherins, Carrier Proteins, Cell Line, Cell Membrane, Cells, Cultured, Cysteine Endopeptidases, Cytoplasm, Dose-Response Relationship, Drug, Down-Regulation, Endopeptidases, Genes, Dominant, Genetic Vectors, Humans, Immunoblotting, Membrane Proteins, Mice, Microscopy, Fluorescence, Multienzyme Complexes, Mutation, Neurons, Peptides, Precipitin Tests, Presenilin-1, Proteasome Endopeptidase Complex, Protein Binding, Protein Structure, Tertiary, Reverse Transcriptase Polymerase Chain Reaction, Subcellular Fractions, Synapses, Time Factors, Transcription, Genetic, Transcriptional Activation, Tubulin
Cell
Date: Sep. 05, 2003
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