TY - JOUR
T1 - The interplay between mitochondrial functionality and genome integrity in the prevention of human neurologic diseases
AU - D'Errico, Mariarosaria
AU - Parlanti, Eleonora
AU - Pascucci, Barbara
AU - Filomeni, Giuseppe
AU - Mastroberardino, Pier Giorgio
AU - Dogliotti, Eugenia
N1 - Funding Information:
The research presented in this review has been partially funded by the PRIN-MIUR grant N° 20152CB22L . We are grateful to Valentina Nanni for graphical work and Annalisa Masi for drawing chemical structures.
Publisher Copyright:
© 2021 Elsevier Inc.
PY - 2021/10/15
Y1 - 2021/10/15
N2 - As mitochondria are vulnerable to oxidative damage and represent the main source of reactive oxygen species (ROS), they are considered key tuners of ROS metabolism and buffering, whose dysfunction can progressively impact neuronal networks and disease. Defects in DNA repair and DNA damage response (DDR) may also affect neuronal health and lead to neuropathology. A number of congenital DNA repair and DDR defective syndromes, indeed, show neurological phenotypes, and a growing body of evidence indicate that defects in the mechanisms that control genome stability in neurons acts as aging-related modifiers of common neurodegenerative diseases such as Alzheimer, Parkinson's, Huntington diseases and Amyotrophic Lateral Sclerosis. In this review we elaborate on the established principles and recent concepts supporting the hypothesis that deficiencies in either DNA repair or DDR might contribute to neurodegeneration via mechanisms involving mitochondrial dysfunction/deranged metabolism.
AB - As mitochondria are vulnerable to oxidative damage and represent the main source of reactive oxygen species (ROS), they are considered key tuners of ROS metabolism and buffering, whose dysfunction can progressively impact neuronal networks and disease. Defects in DNA repair and DNA damage response (DDR) may also affect neuronal health and lead to neuropathology. A number of congenital DNA repair and DDR defective syndromes, indeed, show neurological phenotypes, and a growing body of evidence indicate that defects in the mechanisms that control genome stability in neurons acts as aging-related modifiers of common neurodegenerative diseases such as Alzheimer, Parkinson's, Huntington diseases and Amyotrophic Lateral Sclerosis. In this review we elaborate on the established principles and recent concepts supporting the hypothesis that deficiencies in either DNA repair or DDR might contribute to neurodegeneration via mechanisms involving mitochondrial dysfunction/deranged metabolism.
UR - http://www.scopus.com/inward/record.url?scp=85111250573&partnerID=8YFLogxK
U2 - 10.1016/j.abb.2021.108977
DO - 10.1016/j.abb.2021.108977
M3 - Review article
C2 - 34174223
AN - SCOPUS:85111250573
SN - 0003-9861
VL - 710
JO - Archives of Biochemistry and Biophysics
JF - Archives of Biochemistry and Biophysics
M1 - 108977
ER -
