Open AccessDéjà vu with a twist: transglutaminases in bioenergetics and transcriptional dysfunction in Huntington's disease
Author(s) -
KazemiEsfarjani Parsa,
La Spada Albert R.
Publication year - 2010
Publication title -
embo molecular medicine
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 4.923
H-Index - 107
eISSN - 1757-4684
pISSN - 1757-4676
DOI - 10.1002/emmm.201000092
Subject(s) - neurodegeneration , tissue transglutaminase , huntington's disease , bioenergetics , pathogenesis , disease , biology , neuroprotection , microbiology and biotechnology , neuroscience , immunology , enzyme , medicine , biochemistry , mitochondrion
Abstract The article by McConoughey et al in the current issue of EMBO Molecular Medicine examines the contribution of transglutaminase 2 (TG2) to Huntington's disease (HD) pathogenesis. The authors find that TG2 inhibition can ameliorate HD neurodegeneration, and thereby elevate the status of transglutaminases (TGs) to a major therapeutic target—not because of their well‐known activity in mutant protein aggregation, but instead based upon their ability to epigenetically modulate transcription and energy production. While the reintroduction of TG inhibition as a therapy for HD may evoke feelings of déjà vu , the outcome this time around could go in a dramatically different direction. See related article in EMBO Mol Med (Stephen J. McConoughey et al. (2010) EMBO Mol Med 2: 349–370 )