Beata M. Gruber, Elżbieta L. Anuszewska

Choroba Huntingtona – znaczenie diety we wspomaganiu terapii

Huntington’s disease – the role of a diet as the support for therapy

Huntington’s disease (HD) is a neurodegenerative disorder caused by expansion of a trinucleotide repeat (CAG) in exon 1 of the gene coding for the huntingtin protein. The agglomeration of the mutated huntingtin implicates the disturbances in cellular metabolism and progressive neuronal death. Unlike the other neurodegenerative diseases HD patients even receiving an adequate food intake, show a progressively catabolic state. Some authors suggest that the leading cause of the deaths noted among HD patients is nutritional deficiency, especially vitamin deficits. It was presented the unknown role of the well known chemical compounds, like: amino acids, vitamins or fatty acids as well as the compounds having the defined function as the antioxidants or ergogens. The pre-clinical and clinical data obtained with animal HD models and HD patients are promising for at least reducing the symptoms of HD. Such support for therapy based on the dietary components could have been relatively simple and easy available.
Keywords: Huntington disease, dietary components, energetic metabolism.