On dendrites in Down syndrome and DS murine models: A spiny way to learn

R. Benavides-Piccione, I. Ballesteros-Yáñez, M. Martínez De Lagrán, G. Elston, Xavier P. Estivill, C. Fillat, J. Defelipe, M. Dierssen

Research output: Contribution to journalReview article

85 Citations (Scopus)


Since the discovery in the 1970s that dendritic abnormalities in cortical pyramidal neurons are the most consistent pathologic correlate of mental retardation, research has focused on how dendritic alterations are related to reduced intellectual ability. Due in part to obvious ethical problems and in part to the lack of fruitful methods to study neuronal circuitry in the human cortex, there is little data about the microanatomical contribution to mental retardation. The recent identification of the genetic bases of some mental retardation associated alterations, coupled with the technology to create transgenic animal models and the introduction of powerful sophisticated tools in the field of microanatomy, has led to a growth in the studies of the alterations of pyramidal cell morphology in these disorders. Studies of individuals with Down syndrome, the most frequent genetic disorder leading to mental retardation, allow the analysis of the relationships between cognition, genotype and brain microanatomy. In Down syndrome the crucial question is to define the mechanisms by which an excess of normal gene products, in interaction with the environment, directs and constrains neural maturation, and how this abnormal development translates into cognition and behaviour. In the present article we discuss mainly Down syndrome-associated dendritic abnormalities and plasticity and the role of animal models in these studies. We believe that through the further development of such approaches, the study of the microanatomical substrates of mental retardation will contribute significantly to our understanding of the mechanisms underlying human brain disorders associated with mental retardation.

Original languageEnglish
Pages (from-to)111-126
Number of pages16
JournalProgress in Neurobiology
Issue number2
Publication statusPublished - Oct 2004
Externally publishedYes



  • amyloid beta A4 precursor
  • APP
  • calcium binding protein beta
  • Down syndrome
  • Down syndrome cell adhesion molecule
  • DS
  • GART
  • PCP4
  • phosphoribosylglycinamideformyltransferase
  • Purkinje cell protein 4
  • S100B
  • SIM2
  • single-minded homolog 2
  • SOD1

ASJC Scopus subject areas

  • Neuroscience(all)

Cite this

Benavides-Piccione, R., Ballesteros-Yáñez, I., Martínez De Lagrán, M., Elston, G., Estivill, X. P., Fillat, C., Defelipe, J., & Dierssen, M. (2004). On dendrites in Down syndrome and DS murine models: A spiny way to learn. Progress in Neurobiology, 74(2), 111-126. https://doi.org/10.1016/j.pneurobio.2004.08.001