The DNA damage response: making it safe to play with knives

A Ciccia, SJ Elledge - Molecular cell, 2010‏ - cell.com
Damage to our genetic material is an ongoing threat to both our ability to faithfully transmit
genetic information to our offspring as well as our own survival. To respond to these threats …

DNA interstrand crosslink repair and cancer

AJ Deans, SC West - Nature reviews cancer, 2011‏ - nature.com
Interstrand crosslinks (ICLs) are highly toxic DNA lesions that prevent transcription and
replication by inhibiting DNA strand separation. Agents that induce ICLs were one of the …

Identification of genetic factors that modify clinical onset of Huntington's disease

JM Lee, VC Wheeler, MJ Chao, JPG Vonsattel… - Cell, 2015‏ - cell.com
As a Mendelian neurodegenerative disorder, the genetic risk of Huntington's disease (HD) is
conferred entirely by an HTT CAG repeat expansion whose length is the primary …

A nuclease that mediates cell death induced by DNA damage and poly (ADP-ribose) polymerase-1

Y Wang, R An, GK Umanah, H Park, K Nambiar… - Science, 2016‏ - science.org
INTRODUCTION Poly (ADP-ribose)(PAR) polymerase-1 (PARP-1) is a nuclear enzyme
responding to oxidative stress and DNA damage. Excessive activation of PARP-1 causes an …

DNA repair mechanisms in cancer development and therapy

A Torgovnick, B Schumacher - Frontiers in genetics, 2015‏ - frontiersin.org
DNA damage has been long recognized as causal factor for cancer development. When
erroneous DNA repair leads to mutations or chromosomal aberrations affecting oncogenes …

Fanconi anaemia and the repair of Watson and Crick DNA crosslinks

MC Kottemann, A Smogorzewska - Nature, 2013‏ - nature.com
The function of Fanconi anaemia proteins is to maintain genomic stability. Their main role is
in the repair of DNA interstrand crosslinks, which, by covalently binding the Watson and the …

Ubiquitin-binding proteins: decoders of ubiquitin-mediated cellular functions

K Husnjak, I Dikic - Annual review of biochemistry, 2012‏ - annualreviews.org
Ubiquitin acts as a versatile cellular signal that controls a wide range of biological processes
including protein degradation, DNA repair, endocytosis, autophagy, transcription, immunity …

Exome sequencing of individuals with Huntington's disease implicates FAN1 nuclease activity in slowing CAG expansion and disease onset

B McAllister, J Donaldson, CS Binda, S Powell… - Nature …, 2022‏ - nature.com
The age at onset of motor symptoms in Huntington's disease (HD) is driven by HTT CAG
repeat length but modified by other genes. In this study, we used exome sequencing of 683 …

Dynamics of DNA damage response proteins at DNA breaks: a focus on protein modifications

SE Polo, SP Jackson - Genes & development, 2011‏ - genesdev.cshlp.org
Genome integrity is constantly monitored by sophisticated cellular networks, collectively
termed the DNA damage response (DDR). A common feature of DDR proteins is their …

Postreplicative mismatch repair

J Jiricny - Cold Spring Harbor perspectives in biology, 2013‏ - cshperspectives.cshlp.org
The mismatch repair (MMR) system detects non-Watson–Crick base pairs and strand
misalignments arising during DNA replication and mediates their removal by catalyzing …