Altered mossy fiber distributions in adult Fmr1 (FVB) knockout mice

Hippocampus. 2002;12(1):47-54. doi: 10.1002/hipo.10004.

Abstract

The fragile-X mental retardation protein (FMRP) is greatly reduced or absent in individuals with fragile-X mental retardation syndrome, a common, heritable form of mental retardation. Morphological studies suggest that this protein functions in normal synapse maturation and neuronal plasticity. Examination of human brain autopsy tissue has shown that fragile-X patients exhibit long, thin spines more frequently, and stubby mushroom-shaped spines less frequently, than these two types of spines are seen in normal autopsy tissue. Fragile-X tissue also has a greater density of these spines along dendrites, which suggests a possible failure of synapse elimination. Fmr1 knockout mice and wild-type littermates brains were processed for Timm staining, which reveals the zinc-rich terminals of the dentate gyrus, the mossy fibers. The Fmr1 knockout mice exhibited a pattern of Timm granule-staining within the stratum oriens of subfield CA3 and the inner molecular layer that was significantly different than staining seen in wild-type animals. The sources and consequences of the altered terminal staining are unclear, but are discussed in relation to immature synapse morphology, a failure of normal regression of synapses, and a potential biological penalty of such a failure to regress.

Publication types

  • Research Support, Non-U.S. Gov't
  • Research Support, U.S. Gov't, P.H.S.

MeSH terms

  • Animals
  • Disease Models, Animal
  • Fragile X Mental Retardation Protein
  • Fragile X Syndrome / genetics*
  • Fragile X Syndrome / pathology*
  • Humans
  • Male
  • Mice
  • Mice, Knockout
  • Mossy Fibers, Hippocampal / pathology*
  • Nerve Tissue Proteins / deficiency*
  • Nerve Tissue Proteins / genetics*
  • Nerve Tissue Proteins / physiology
  • RNA-Binding Proteins*
  • Staining and Labeling
  • Synapses / pathology

Substances

  • FMR1 protein, human
  • Fmr1 protein, mouse
  • Nerve Tissue Proteins
  • RNA-Binding Proteins
  • Fragile X Mental Retardation Protein