Piacentini, M.

disease protein huntington autophagy transglutaminase death activity cell death mitochondrial apoptosi tissue tissue transglutaminase function enzyme journal medicine fi ndings medicine journal mitochondria piacentini pathogenesis level neurodegenerative diseases neurodegenerative mechanism type 2 transglutaminase calcium mastroberardino model huntingtin study nding effect result polyq neuron intracellular cross-linking receptor tg 2 inhibition association brain aggregate regulation publication autophagosome review-symposium 419–431 farrace mg ª 2010 fication process presence piacentini m substrate degradation expression patient inhibition activation kinase transamidation defect cross-linking activity mu-htt pdi activity treatment huntington disease fi nding tg 2. transamidation activity pathway symptom grif fin m localization polyglutamine condition neurodegeneration animal martin jb