[Model of Huntington's Disease Induced with 3-nitropropionic Acid]

Overview

Journal Rev Neurol

Specialty Neurology

Date 2009 Apr 3

PMID 19340784

Citations 8

Authors

I Tunez

A Santamaria

Affiliations

Soon will be listed here.

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.

Citing Articles

Extremely low frequency electromagnetic fields stimulation modulates autoimmunity and immune responses: a possible immuno-modulatory therapeutic effect in neurodegenerative diseases.

Guerriero F, Ricevuti G Neural Regen Res. 2017; 11(12):1888-1895.

PMID: 28197174 PMC: 5270416. DOI: 10.4103/1673-5374.195277.

Ameliorating effect of Celastrus paniculatus standardized extract and its fractions on 3-nitropropionic acid induced neuronal damage in rats: possible antioxidant mechanism.

Malik J, Karan M, Dogra R Pharm Biol. 2017; 55(1):980-990.

PMID: 28164735 PMC: 6130694. DOI: 10.1080/13880209.2017.1285945.

Neuronal cellular responses to extremely low frequency electromagnetic field exposure: implications regarding oxidative stress and neurodegeneration.

Reale M, Kamal M, Patruno A, Costantini E, DAngelo C, Pesce M PLoS One. 2014; 9(8):e104973.

PMID: 25127118 PMC: 4134243. DOI: 10.1371/journal.pone.0104973.

Puerarin ameliorates 3-nitropropionic acid-induced neurotoxicity in rats: possible neuromodulation and antioxidant mechanisms.

Mahdy H, Mohamed M, Emam M, Karim A, Abdel-Naim A, Khalifa A Neurochem Res. 2013; 39(2):321-32.

PMID: 24346712 DOI: 10.1007/s11064-013-1225-7.

3-Nitropropionic acid as a tool to study the mechanisms involved in Huntington's disease: past, present and future.

Tunez I, Tasset I, Perez-De la Cruz V, Santamaria A Molecules. 2010; 15(2):878-916.

PMID: 20335954 PMC: 6263191. DOI: 10.3390/molecules15020878.