title: Type 2 Transglutaminase, mitochondria and Huntington's disease: menage a trois.
abstract: Mitochondria produce the bulk of cellular energy and work as decisional "hubs" for cellular responses by integrating different input signals. The determinant in the physiopathology of mammals, they attract major attention, nowadays, for their contribution to brain degeneration. How they can withstand or succumb to insults leading to neuronal death is an object of great attention increasing the need for a better understanding of the interplay between inner and outer mitochondrial pathways residing in the cytosol. Of the latter, those dictating protein metabolism and therefore influencing the quality function and control of the organelle are of our most immediate interest and here we describe the Transglutaminase type 2 (TG2) contribution to mitochondrial function, dysfunction and neurodegeneration. Besides reviewing the latest evidences we share also the novel ones on the IF1 pathway depicting a molecular conduit governing mitochondrial turnover and homeostasis relevant to envisaging preventive and therapeutic strategies to respectively predict and counteract deficiencies associated with deregulated mitochondrial function in neuropathology.
authors.affiliation: Department of Biology, University of Rome 'Tor Vergata', Rome 00133, Italy.
authors.forename: Sara
authors.initials: S
authors.lastname: Altuntas
authors.affiliation: Department of Biology, University of Rome 'Tor Vergata', Rome 00133, Italy.
authors.forename: Manuela
authors.initials: M
authors.lastname: D'Eletto
authors.affiliation: Department of Biology, University of Rome 'Tor Vergata', Rome 00133, Italy.
authors.forename: Federica
authors.initials: F
authors.lastname: Rossin
authors.affiliation: Department of Biology, University of Rome 'Tor Vergata', Rome 00133, Italy.
authors.forename: Laura Diaz
authors.initials: LD
authors.lastname: Hidalgo
authors.affiliation: Department of Biology, University of Rome 'Tor Vergata', Rome 00133, Italy.
authors.forename: Maria Grazia
authors.initials: MG
authors.lastname: Farrace
authors.affiliation: National Institute for Infectious Diseases I.R.C.C.S. 'L. Spallanzani', Rome 00149, Italy.
authors.forename: Laura
authors.initials: L
authors.lastname: Falasca
authors.affiliation: Department of Biology, University of Rome 'Tor Vergata', Rome 00133, Italy.
authors.forename: Lucia
authors.initials: L
authors.lastname: Piredda
authors.affiliation: European Brain Research Institute (EBRI), Rita Levi-Montalcini Foundation, Rome 00143, Italy.
authors.forename: Stefania
authors.initials: S
authors.lastname: Cocco
authors.affiliation: Department of Genetics, Erasmus Medical Center, Rotterdam 3015, The Netherlands.
authors.forename: Pier Giorgio
authors.initials: PG
authors.lastname: Mastroberardino
authors.affiliation: Department of Biology, University of Rome 'Tor Vergata', Rome 00133, Italy; National Institute for Infectious Diseases I.R.C.C.S. 'L. Spallanzani', Rome 00149, Italy. Electronic address: Mauro.piacentini@uniroma2.it.
authors.forename: Mauro
authors.initials: M
authors.lastname: Piacentini
authors.affiliation: Department of Biology, University of Rome 'Tor Vergata', Rome 00133, Italy; European Brain Research Institute (EBRI), Rita Levi-Montalcini Foundation, Rome 00143, Italy; Department of Comparative Biomedical Sciences, Royal Veterinary College, University of London, UCL Consortium for Mitochondrial Research (CfMR), London, NW1 0TU, UK. Electronic address: mcampanella@rvc.ac.uk.
authors.forename: Michelangelo
authors.initials: M
authors.lastname: Campanella
mesh_terms: D004734:Energy Metabolism; D019204:GTP-Binding Proteins / Q000235:genetics / Q000378:metabolism*; D015971:Gene Expression Regulation, Enzymologic; D006801:Humans; D006816:Huntington Disease / Q000235:genetics / Q000378:metabolism*; D008928:Mitochondria / Q000378:metabolism*; D000091345:Protein Glutamine gamma Glutamyltransferase 2; D011503:Transglutaminases / Q000235:genetics / Q000378:metabolism*
chemical_list: D000091345:Protein Glutamine gamma Glutamyltransferase 2; D011503:Transglutaminases; D019204:GTP-Binding Proteins
keywords: IF(1); Mitochondria; Mitophagy; Neurodegeneration; TG2