[Model of Huntington's disease induced with 3-nitropropionic acid].

Abstract

INTRODUCTION:

Huntington's disease is an autosomal dominant hereditary disorder. This neurodegenerative illness is characterized by mutation of the huntingtin protein gene, causing the formation of intracellular protein aggregates.

DEVELOPMENT:

Intensive research efforts have been made to investigate the molecular mechanism involved. For this reason, the development of animal and cellular models of Huntington's disease has offered alternative approaches to study of this disease. The alteration of succinate dehydrogenase activity has been linked to Huntington's disease. 3-nitropropionic acid is an inhibitor of this enzyme, prompting oxidative stress and death neuronal, mimic some aspects of Huntington's disease as anatomical, physiological and chemical changes.

CONCLUSION:

This model is a useful tool to study the mechanisms involved in this disease and to evaluate new therapeutic strategies.

• Authors: I Túnez, A Santamaría
• Journal: Revista de neurologia (Rev Neurol) 2009 Apr 16-30 Vol. 48 Issue 8 Pg. 430-4 ISSN: 1576-6578 [Electronic] Spain
• Vernacular Title: Modelo de enfermedad de Huntington inducido con ácido 3-nitropropiónico.
• PMID: 19340784 (Publication Type: Journal Article, Review)

Chemical References

• Convulsants
• HTT protein, human
• Huntingtin Protein
• Nerve Tissue Proteins
• Neurotoxins
• Nitro Compounds
• Nuclear Proteins
• Propionates
• Succinate Dehydrogenase
• 3-nitropropionic acid

Topics

• Animals
• Convulsants (metabolism, toxicity)
• Disease Models, Animal
• Humans
• Huntingtin Protein
• Huntington Disease (chemically induced, physiopathology)
• Nerve Tissue Proteins (genetics, metabolism)
• Neurotoxins (metabolism, toxicity)
• Nitro Compounds (metabolism, toxicity)
• Nuclear Proteins (genetics, metabolism)
• Propionates (metabolism, toxicity)
• Succinate Dehydrogenase (antagonists & inhibitors, metabolism)