Rho-linked genes and neurological disorders
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  • 作者:Nael Nadif Kasri (1)
    Linda Van Aelst (1)
  • 关键词:Mental retardation ; Dendritic spines ; Actin cytoskeleton ; Rho GTPases ; X ; linked mental retardation ; Amyotrophic lateral sclerosis
  • 刊名:Pfl眉gers Archiv - European Journal of Physiology
  • 出版年:2008
  • 出版时间:February 2008
  • 年:2008
  • 卷:455
  • 期:5
  • 页码:787-797
  • 全文大小:312KB
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  • 作者单位:Nael Nadif Kasri (1)
    Linda Van Aelst (1)

    1. Cold Spring Harbor Laboratory, 1 Bungtown Road, Demerec Building, Cold Spring Harbor, NY, 11724, USA
  • ISSN:1432-2013
文摘
Mental retardation (MR) is a common cause of intellectual disability and affects approximately 2 to 3% of children and young adults. Many forms of MR are associated with abnormalities in dendritic structure and/or dendritic spine morphology. Given that dendritic spine morphology has been tightly linked to synaptic activity, altered spine morphology has been suggested to underlie or contribute to the cognitive disabilities associated with MR. The structure and dynamics of dendritic spines is determined by its underlying actin cytoskeleton. Signaling molecules and cascades important for cytoskeletal regulation have therefore attracted a great deal of attention. As key regulators of both the actin and microtubule cytoskeletons, it is not surprising that the Rho GTPases have emerged as important regulators of dendrite and spine structural plasticity. Significantly, mutations in regulators and effectors of Rho GTPases have been associated with diseases affecting the nervous system, including MR and amyotropic lateral sclerosis (ALS). Here, we will discuss Rho GTPase-related genes and their signaling pathways involved in MR and ALS.

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