Animal models of mental retardation: from gene to cognitive function.

Abstract:

:About 2-3% of all children are affected by mental retardation, and genetic conditions rank among the leading causes of mental retardation. Alterations in the information encoded by genes that regulate critical steps of brain development can disrupt the normal course of development, and have profound consequences on mental processes. Genetically modified mouse models have helped to elucidate the contribution of specific gene alterations and gene-environment interactions to the phenotype of several forms of mental retardation. Mouse models of several neurodevelopmental pathologies, such as Down and Rett syndromes and X-linked forms of mental retardation, have been developed. Because behavior is the ultimate output of brain, behavioral phenotyping of these models provides functional information that may not be detectable using molecular, cellular or histological evaluations. In particular, the study of ontogeny of behavior is recommended in mouse models of disorders having a developmental onset. Identifying the role of specific genes in neuropathologies provides a framework in which to understand key stages of human brain development, and provides a target for potential therapeutic intervention.

journal_name

Neurosci Biobehav Rev

authors

Branchi I,Bichler Z,Berger-Sweeney J,Ricceri L

doi

10.1016/s0149-7634(03)00016-2

subject

Has Abstract

pub_date

2003-01-01 00:00:00

pages

141-53

issue

1-2

eissn

0149-7634

issn

1873-7528

pii

S0149763403000162

journal_volume

27

pub_type

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