Disruption of PLC-beta 1-mediated signal transduction in mutant mice causes age-dependent hippocampal mossy fiber sprouting and neurodegeneration

Mol Cell Neurosci. 2002 Dec;21(4):584-601. doi: 10.1006/mcne.2002.1199.

Abstract

Aberrant reorganization of hippocampal mossy fibers occurs in human temporal lobe epilepsy and rodent epilepsy models. We generated a mouse model showing massive late-onset aberrant mossy fiber sprouting in the adult hippocampus. The mutation in this mouse model derives from an intronic insertion of transgene DNA in the mouse PLC-beta1 gene (PLC-beta 1(-/-)(TC) mutation) leading to a splice mutation of the PLC-beta 1 gene and a complete loss of downstream PLC-beta 1 expression. PLC-beta 1(-/-)(TC) mutants develop a loss of NMDA-receptors in the stratum oriens of region CA1, apoptotic neuronal death, and reduced hippocampal PKC activity. The phenotype of these mice further consists of a late-onset epileptiform hyperexcitability, behavioral modifications in a radial maze and in an open field, female nurturing defect, and male infertility. In the present study, we provide evidence that the arising of the behavioral phenotype in PLC-beta 1(-/-)(TC) mice correlates in time with the development of the aberrant mossy fiber projections and that the disruption of the PLC-beta 1-mediated signal transduction pathway may lead to a functional cholinergic denervation, which could cause hippocampal remodeling and, in consequence, epileptiform hyperexcitability.

MeSH terms

  • Aging / genetics*
  • Aging / metabolism
  • Alternative Splicing / genetics
  • Animals
  • Carbachol / pharmacology
  • Chromosome Mapping
  • Disease Models, Animal
  • Epilepsy, Temporal Lobe / enzymology
  • Epilepsy, Temporal Lobe / genetics*
  • Epilepsy, Temporal Lobe / physiopathology
  • Female
  • Growth Cones / enzymology
  • Isoenzymes / deficiency*
  • Isoenzymes / genetics
  • Male
  • Mice
  • Mice, Knockout
  • Mice, Neurologic Mutants
  • Mossy Fibers, Hippocampal / enzymology*
  • Mossy Fibers, Hippocampal / pathology
  • Mutation / genetics
  • Nerve Degeneration / enzymology
  • Nerve Degeneration / genetics*
  • Nerve Degeneration / physiopathology
  • Neuronal Plasticity / genetics
  • Phospholipase C beta
  • Protein Kinase C / metabolism
  • Receptors, N-Methyl-D-Aspartate / metabolism
  • Recombinant Fusion Proteins / genetics
  • Signal Transduction / genetics*
  • Transgenes / genetics
  • Type C Phospholipases / deficiency*
  • Type C Phospholipases / genetics

Substances

  • Isoenzymes
  • Receptors, N-Methyl-D-Aspartate
  • Recombinant Fusion Proteins
  • Carbachol
  • Protein Kinase C
  • Type C Phospholipases
  • PLCB1 protein, human
  • Phospholipase C beta
  • Plcb1 protein, mouse