Nε‐(γ‐l‐Glutamyl)‐l‐lysine (GGEL) is increased in cerebrospinal fluid of patients with Huntington's disease
- 1 November 2001
- journal article
- research article
- Published by Wiley in Journal of Neurochemistry
- Vol. 79 (5) , 1109-1112
- https://doi.org/10.1046/j.1471-4159.2001.00673.x
Abstract
Pathological-length polyglutamine (Qn) expansions, such as those that occur in the huntingtin protein (htt) in Huntington's disease (HD), are excellent substrates for tissue transglutaminase in vitro, and transglutaminase activity is increased in post-mortem HD brain. However, direct evidence for the participation of tissue transglutaminase (or other transglutaminases) in HD patients in vivo is scarce. We now report that levels of Nε-(γ-l-glutamyl)-l-lysine (GGEL)– a ‘marker’ isodipeptide produced by the transglutaminase reaction – are elevated in the CSF of HD patients (708 ± 41 pmol/mL, SEM, n = 36) vs. control CSF (228 ± 36, n = 27); p < 0.0001. These data support the hypothesis that transglutaminase activity is increased in HD brain in vivo.Keywords
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