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

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.

journal_name

Neurochem Int

authors

Karpuj MV,Becher MW,Steinman L

doi

10.1016/s0197-0186(01)00060-2

subject

Has Abstract

pub_date

2002-01-01 00:00:00

pages

31-6

issue

1

eissn

0197-0186

issn

1872-9754

pii

S0197018601000602

journal_volume

40

pub_type

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