NYMC Faculty Publications

γ-Glutamylamines and Neurodegenerative Diseases

Author Type(s)

Faculty

DOI

10.1007/s00726-011-1209-3

Journal Title

Amino Acids

First Page

129

Last Page

142

Document Type

Article

Publication Date

1-1-2013

Department

Biochemistry and Molecular Biology

Keywords

Amines, Animals, Dipeptides, Glutamine, Humans, Lysine, Neurodegenerative Diseases, Protein Processing, Post-Translational, Transglutaminases, gamma-Glutamylcyclotransferase

Disciplines

Biochemical Phenomena, Metabolism, and Nutrition | Medical Biochemistry | Medicine and Health Sciences

Abstract

Transglutaminases catalyze the formation of γ-glutamylamines utilizing glutamyl residues and amine-bearing compounds such as lysyl residues and polyamines. These γ-glutamylamines can be released from proteins by proteases in an intact form. The free γ-glutamylamines can be catabolized to 5-oxo-L-proline and the free amine by γ-glutamylamine cyclotransferase. Free γ-glutamylamines, however, accumulate in the CSF and affected areas of Huntington Disease brain. This observation suggests transglutaminase-derived γ-glutamylamines may play a more significant role in neurodegeneration than previously thought. The following monograph reviews the metabolism of γ-glutamylamines and examines the possibility that these species contribute to neurodegeneration.

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