Transglutaminases and Neurological Diseases

  • Julianne Feola
  • Alina Monteagudo
  • Laura Yunes-Medina
  • Gail V. W. Johnson
Chapter
First Online:

Abstract

Transglutaminases, and predominantly transglutaminase 2 (TG2), have been implicated in the pathogenesis of a number of neurological conditions. In the context of older-age onset neurodegenerative diseases that are characterized by protein aggregates, it was initially postulated that transglutaminases contributed to the pathogenic process by crosslinking and facilitating aggregate formation. However, more recent studies have provided evidence that although TG2, and possibly other transglutaminases, likely contribute to the progression of certain neurodegenerative diseases, this probably does not involve crosslinking and the formation of insoluble protein aggregates. Indeed, there is growing evidence that TG2 could be a contributing factor in neurological diseases through mechanisms such as modulating transcriptional events, affecting cell migration and adhesion, or altering cellular differentiation processes. In this chapter, neurological diseases in which transglutaminases have been implicated as a contributing factor are discussed. The chapter will conclude with a discussion about cystamine, a non-specific in vitro inhibitor of transglutaminases, as a neuroprotective agent.

Keywords

Transglutaminase 2 Neurological disease Cystamine Transcription Adhesion Migration Proliferation 
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Copyright information

© Springer Japan 2015

Authors and Affiliations

  • Julianne Feola
    • 1
  • Alina Monteagudo
    • 2
  • Laura Yunes-Medina
    • 1
  • Gail V. W. Johnson
    • 3
    • 2
    • 1
  1. 1.Neuroscience ProgramUniversity of RochesterRochesterUSA
  2. 2.Department of Pharmacology and PhysiologyUniversity of RochesterRochesterUSA
  3. 3.Department of AnesthesiologyUniversity of RochesterRochesterUSA

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