TRANSGLUTAMINASE ACTIVITY IS RELATED TO CAG REPEAT LENGTH IN PATIENTSWITH HUNTINGTONS-DISEASE

Huntington's disease (HD) is a neurodegenerative disorder associated w ith CAG repeat expansion, We measured transglutaminase (TGase) activit y in lymphocytes from 35 HD patients and from healthy individuals to a scertain whether it was altered in this condition, TGase activity was above maximum control levels in 25% of HD patients; it was correlated with the age of the patient and inversely correlated with the CAG repe at length. These results suggest that: (1) HD could be biochemically h eterogeneous, and (2) the length of the CAG repeat expansion/TGase rat io could be important in the manifestation of HD.