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Reviewing Biochemical Implications of Normal and Mutated Huntingtin in Huntington's Disease

Author(s):

Ester Tellone*, Antonio Galtieri and Silvana Ficarra   Pages 1 - 21 ( 21 )

Abstract:


Huntingtin (Htt) is a multi-function protein of the brain. Normal Htt shows a common alpha-helical structure but conformational changes into a form with beta strands is the principal cause of Huntington's disease." Huntington's disease is a genetic neurological disorder caused by a repeated expansion of the CAG trinucleotide instability in the N-terminal of the gene coding for the Huntingtin protein. The mutation leads to the abnormal expansion of the production of the polyglutamine tract (polyQ) resulting in the form of an unstable Huntingtin protein commonly referred to as mutant Huntingtin. Mutant Huntingtin is the cause of the complex neurological metabolic alteration of Huntington's disease both for the loss of all the functions of normal Huntingtin and for the genesis of abnormal interactions due to the presence of this mutation. One of the problems arising from the misfolded Huntingtin is the increase in oxidative stress, which is common to many neurological diseases such as Alzheimer's, Parkinson's, Amyotrophic Lateral Sclerosis and Creutzfeldt–Jakob disease. In the last few years, use of antioxidants, had a strong incentive to find valid therapies to the defence against neurodegenerations. Although further studies are needed, the use of antioxidant mixtures to counteract neuronal damages seems promising.

Keywords:

Huntington`s disease, Neurodegeneration, Misfolding, Antioxidants, Oxidative stress

Affiliation:

Department of Chemical, Biological, Pharmaceutical and Environmental Sciences, University of Messina, V.le Ferdinando Stagno d`Alcontres 31, 98166 Messina, Department of Chemical, Biological, Pharmaceutical and Environmental Sciences, University of Messina, V.le Ferdinando Stagno d`Alcontres 31, 98166 Messina, Department of Chemical, Biological, Pharmaceutical and Environmental Sciences, University of Messina, V.le Ferdinando Stagno d`Alcontres 31, 98166 Messina



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