| Sumario: | ABS TRACT Hun ting ton's Di se a se is an au to so mal do mi nant, la te on set and fa tal di sor der cha rac - te ri zed by in vo lun tary cho re ic mo ve ments, cog ni ti ve dysfunc ti on and psycho lo gi cal dis tur ban ces. The ca u sa ti ve mu ta ti on is the ex pan si on of the CAG re pe ats in the first exon of the IT-15 ge ne. Mu tant ge ne ex pres si on ca u ses se lec ti ve de ath of stri a tal gam ma ami no but yric acid re le a sing (GA BA-er gic) me di um si ze spiny ne u rons. Chan ges in the con for ma ti on of the mu tant hun ting tin (htt) pro te in, pro te oly sis and ubi qu i tin-pro te a so me system (UPS), trans crip ti o nal dis re gu la ti on and ex ci to to xi city are among the mo le cu lar mec ha nisms of ne u ro de ge ne ra ti on. Ex cess amo unts of exci ta tory che mi cals con ti nu o usly sti mu la te the cells and ca u se ex ci to to xic de ath of vul ne rab le cells. Glu ta ma te is one of the ex ci ta tory ne u rot rans mit ters re le a sed from ba sal gang li a. The stri a tal ne u - ron loss in Hun ting ton's Di se a se po ints out that glu ta ma te re cep tor me di a ted ex ci to to xi city may play a ro le in the pat ho ge ne sis. Ex ci ta ti on of N-methyl-D-As par ta te re cep tors (NMDARs), which be long to io not rop hic glu ta ma te re cep tors (iG luR), fol lo wed by ac ti va ti on of nuc le ar fac tor kap pa B (NF ± B) trans crip ti on fac tor re sult in ne u ro nal de ath. NMDARs carry pol ya mi ne bin ding si tes apart from glu ta ma te bin ding si tes. Sin ce pol ya mi nes are in con tact with the mu tant htt pro te in, NMDARs and NF ± B trans crip ti on fac tor, they may play a cen tral ro le in ne u ro de ge ne ra ti on ob ser ved in Hun ting ton's Di se a se. Thus, de ta i led in ves ti ga ti on and un ders tan ding of the pol ya mi ne me ta bo - lism in Hun ting ton's Di se a se will pro vi de new ap pro ac hes in the tre at ment of the di se a se.
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