Amino Acids

, Volume 26, Issue 4, pp 373–379

The multifaceted role of transglutaminase in neurodegeneration: Review article

  • M. Karpuj
  • L. Steinman

DOI: 10.1007/s00726-004-0082-8

Cite this article as:
Karpuj, M. & Steinman, L. Amino Acids (2004) 26: 373. doi:10.1007/s00726-004-0082-8


A critical role for transglutaminase [TGase] has been hypothesized in the pathogenesis of the CAG trinucleotide repeat diseases, characterized by proteins with abnormal expansions of a polyglutamine domain. In the last few years the involvement of TGase in neurodegenerative diseases [NDS], including its role in aggregate formation, has been broadened to include Alzheimer’s [AD] and Parkinson’s Disease [PD]. It is clear that reduction of TGase activity is beneficial for prolonged survival in mouse models of NDS. The pathological progression of these diseases might reflect in part increases of TGase induced aggregates, or changes in other pathways influenced by increases in TGase activity. Neurodegeneration may be influenced by increased TGase activity affecting apoptosis, modulation of GTPase activity and signal transduction. This review will focus on the leading hypotheses in relation to both old and new experimental results.

Keywords: Transglutaminase – Neurodegeneration – Huntington disease – Alzheimer’s disease – Parkinson’s disease – Cystamine

Copyright information

© Springer-Verlag/Wien 2004

Authors and Affiliations

  • M. Karpuj
    • 1
  • L. Steinman
    • 2
  1. 1.Institute for Neurodegenerative Diseases, University of CaliforniaSan FranciscoU.S.A.
  2. 2.Department of Neurological Sciences and DirectorInterdepartmental Program in Immunology, Stanford UniversityStanfordUSA