N <sup><nobr> ε </nobr></sup>-(γ-l-Glutamyl)-l-lysine (GGEL) is increased in cerebrospinal fluid of patients with Huntington's disease期刊界 All Journals 搜尽天下杂志传播学术成果专业期刊搜索期刊信息化学术搜索

按检索

N ^ε-(γ-l-Glutamyl)-l-lysine (GGEL) is increased in cerebrospinal fluid of patients with Huntington's disease

Authors:	Thomas M Jeitner § Mikhail B Bogdanov Wayne R Matson ¶ Yevgeny Daikhin Marc Yudkoff John E Folk †† Lawrence Steinman ‡‡ Susan E Browne M Flint Beal John P Blass†§ Arthur J L Cooper‡§

Institution:	Department of Neurology and Neuroscience, Weill Medical College of Cornell University, New York, USA.

Abstract:	Pathological-length polyglutamine (Q(n)) 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 N(epsilon)-(gamma-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:	cerebrospinal fluid N ^ε-(γ-l-glutamyl)-l-lysine Huntington's disease neurodegenerative diseases transglutaminases