The molecular and philogenetic aspects of the patogenesis of Huntington’s disease
Keywords:
Хорея Гентингтона, гентингтин, каспаза, апоптоз, нейродегенерація, динамічна мутація, еволюція, ембріональна нейротрансплантація.Abstract
Huntington’s chorea is a demielinisated hereditary disease, which is caused by the dynamic mutation of the huntingtin’s gene (4р16-3), that drives to abnormal (more then 37 repetitions) increasing of GAG-triplets number (encodes glutamine). Polyglutamine tracts of the huntingtin acquire ability to interconnect and to activate caspase-8, which initiates apoptosis in the neurons of the brain structures specific for this disease. The evolutional interpretation of this disease origin, as consequence of necessary hungtingtin mutation of, with a view to the limitation of uncontroled mutations rising, that takes place in forced evolution are proposed. In case of Huntington’s disease, a modern method of restoring injured brain structures is a microsurgical politopic transplantation of the necessary type of partially differentiated (in stem-cells cultures) embryonic cells into demaged structures with further formation of intracerebral intercourses intrinsic to ripe neurons of these structures.
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