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Down syndrome is an oxidative phosphorylation disorder.

M Pilar Bayona-Bafaluy1, Nuria Garrido-Pérez1, Patricia Meade1

  • 1Departamento de Bioquímica, Biología Molecular y Celular, Universidad de Zaragoza, C/ Miguel Servet, 177. 50013, Zaragoza, Spain and C/ Pedro Cerbuna, 12, 50009, Zaragoza, Spain; Instituto de Investigación Sanitaria (IIS) de Aragón, Av. San Juan Bosco, 13, 50009, Zaragoza, Spain; Centro de Investigaciones Biomédicas en Rd de Enfermedades Raras (CIBERER), Av. Monforte de Lemos, 3-5, 28029, Madrid, Spain; Instituto de Biocomputación y Física de Sistemas Complejos (BIFI), Universidad de Zaragoza. C/ Mariano Esquillor (Edificio I+D), 50018, Zaragoza, Spain.

Redox Biology
|February 4, 2021
PubMed
Summary

Down syndrome, caused by trisomy 21, impairs mitochondrial function and neurogenesis early in development. Early therapeutic interventions targeting mitochondrial biogenesis may improve neurogenesis and cognitive outcomes.

Keywords:
Brain developmentDown syndromeMitochondrial biogenesisNeurogenesisOxidative phosphorylation

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Area of Science:

  • Genetics
  • Neuroscience
  • Mitochondrial Biology

Background:

  • Down syndrome, characterized by trisomy 21, is the leading genetic cause of intellectual disability.
  • Genes on chromosome 21 can inhibit mitochondrial biogenesis, impacting cellular energy production.
  • Impaired oxidative phosphorylation is observed early in Down syndrome development, affecting various tissues.

Purpose of the Study:

  • To investigate if early overexpression of chromosome 21 genes impairs prenatal oxidative phosphorylation.
  • To determine the impact of this impairment on neurogenesis.
  • To explore potential therapeutic strategies for Down syndrome.

Main Methods:

  • Analysis of mitochondrial biogenesis and oxidative phosphorylation in prenatal Down syndrome models.
  • Assessment of neurogenesis markers and function.
  • Review of existing and potential therapeutic interventions.

Main Results:

  • Early overexpression of specific genes on chromosome 21 can lead to prenatal mitochondrial dysfunction.
  • This mitochondrial impairment negatively affects neurogenesis.
  • Conditions associated with Down syndrome often overlap with primary oxidative phosphorylation disorders.

Conclusions:

  • Oxidative phosphorylation defects are an early and pervasive event in Down syndrome.
  • Therapeutic strategies aimed at enhancing mitochondrial biogenesis during critical prenatal and neonatal periods are warranted.
  • Improving mitochondrial function may offer a promising avenue for treating neurodevelopmental deficits in Down syndrome.