Fragile X brain synapses mostly undeveloped, researchers say

Views of normal brains and of those afflicted with Fragile X Mental Retardation Syndrome are coming into focus, and the contrast in synapse development is vividly clear, say researchers at the University of Illinois Beckman Institute for Advanced Science and Technology.

A big difference is in dendritic spines--projections from nerve cells through which many impulses make their synaptic connections. Normal brains have thick, well-developed spines; mostly long, narrow and undeveloped spines protrude from the nerve cells in Fragile X brains.

The apparatus for normal development exists in both brains, but the message carrier is not working in Fragile X brains, researchers William T. Greenough and Ivan Jeanne Weiler reported Aug. 25 at the Ninth Annual International Workshop on Fragile X Syndrome and X-Linked Mental Retardation in Strasbourg, France. In Boston, before traveling to Europe, Greenough received a Distinguished Scientific Contribution Award during the American Psychological Association annual convention for his research on the mechanisms underlying learning and memory.

When stimulated in early development, spines in normal brain tissue rapidly record the experience, generate protein, grow and mature, forming a characteristic thick, functional shape. Spines in Fragile X brains don't change or mature. The Fragile X mental retardation protein (FMRP) is not working, thus secondary protein synthesis necessary for maturation does not occur, the researchers reported.

Their work-- funded by the FRAXA Research Foundation and the National Institute of Child Health and Human Development-- involves experiments with mice and rats and examinations of autopsy samples of human Fragile X patients.

In May 1997, a team led by Greenough and Weiler reported that FMRP is produced at synapses of the brain. Genetic suppression of FMRP already was known to cause mental retardation. Fragile X Syndrome is the most common cause of genetically inherited ment

Contact: Jim Barlow, Life Sciences Editor
University of Illinois at Urbana-Champaign

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