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Review
. 2002 Jan;40(1):31-6.
doi: 10.1016/s0197-0186(01)00060-2.

Evidence for a role for transglutaminase in Huntington's disease and the potential therapeutic implications

Marcela V Karpuj  1 , Mark W Becher, Lawrence Steinman
Affiliations
Review

Evidence for a role for transglutaminase in Huntington's disease and the potential therapeutic implications

Marcela V Karpuj et al. Neurochem Int. 2002 Jan.

Abstract

Transglutaminase (TGase) activity is increased in affected regions of brains from patients with Huntington's disease (HD). TGase activity is particularly elevated in the nucleus compared with the cytoplasm from these brains. Gamma-glutaminyl-lysyl cross-links have been detected in nuclear inclusions in HD brain, indicating that TGase may play a prominent role in the aggregation of huntingtin (htt). Attempts to ameliorate experimental disease, via inhibition of TGase in transgenic models of HD in mice, are under investigation.

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