The various activities of TG2 have been directly implicated in the progression of celiac disease, arthrosclerosis, diabetes, cancer, glaucoma and the formation of cataracts [27].Additionally, TG2 has a role in multiple neurodegenerative diseases including Huntington’s (HD) [28], Parkinson’s (PD) as well as Alzheimer’s disease (AD) [29], [30].Studies of patient brains have shown that expression levels and transamidating activity of TG2 are highly elevated in these diseases, where it has been proposed that cell death may be caused by the aberrant cross-linking of substrates [30]. Here, TGM2 is linked to Huntington disease.