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Defective DNA Damage Responses in Dominant Neurodegenerative Diseases

Total Cost €


EC-Contrib. €






 SIDSCA project word cloud

Explore the words cloud of the SIDSCA project. It provides you a very rough idea of what is the project "SIDSCA" about.

genetically    molecular    protein    ataxias    activation    lethality    arising    neurodegenerative    sensor    illustrated    cutting    attenuated    scan1    sporadic    etiological    oculomotor    single    strand    neuropathy    treatment    ultimately    mechanism    mechanisms    breaks    spinocerebellar    threat    sclerosis    licensed    techniques    scas    amyotrophic    embryonic    ataxia    questions    deletion    cells    mcsz    involvement    diseases    axonal    normal    data    disease    neurological    therapeutic    genome    als    survival    ageing    apraxia    ssb    sca    suggest    hereditary    inhibitors    frequent    cellular    ssbs    parp1    again    hypotheses    humans    genetic    ssbr    defective    damage    onset    engage    integrity    proteins    human    explore    dna    motor    examine    microcephaly    mnd    repair    lesions    thereby    trigger    neurone    lateral    indicated    extends    seizures    edge    cancer    therapy    dominant    neurodegeneration    defects    elevated    cell    systematically    rare    aoa1    break    induce   

Project "SIDSCA" data sheet

The following table provides information about the project.


Organization address
postcode: BN1 9RH

contact info
title: n.a.
name: n.a.
surname: n.a.
function: n.a.
email: n.a.
telephone: n.a.
fax: n.a.

 Coordinator Country United Kingdom [UK]
 Project website
 Total cost 2˙447˙409 €
 EC max contribution 2˙447˙409 € (100%)
 Programme 1. H2020-EU.1.1. (EXCELLENT SCIENCE - European Research Council (ERC))
 Code Call ERC-2015-AdG
 Funding Scheme ERC-ADG
 Starting year 2016
 Duration (year-month-day) from 2016-10-01   to  2021-09-30


Take a look of project's partnership.

# participants  country  role  EC contrib. [€] 
1    THE UNIVERSITY OF SUSSEX UK (BRIGHTON) coordinator 1˙783˙034.00


 Project objective

DNA single-strand breaks (SSBs) are the most frequent DNA lesions arising in cells and are a major threat to cell survival and genome integrity, as indicated by the elevated genetic deletion, embryonic lethality, or neurological disease observed if single-strand break repair (SSBR) is attenuated. In particular, SSBR defects are associated with hereditary neurodegeneration in humans, as illustrated by the genetic diseases ataxia oculomotor apraxia-1 (AOA1), spinocerebellar ataxia with axonal neuropathy-1 (SCAN1), and microcephaly with early onset seizures (MCSZ). However, two major questions remain: what are the mechanisms by which SSBs trigger neurodegeneration, and to what extent do SSBs contribute to other genetic and/or sporadic neurodegenerative disease? Based on exciting new data we now propose that the impact of SSBs on neurodegeneration extends beyond rare SSBR-defective diseases to include more common motor neurone diseases (amyotrophic lateral sclerosis) and the genetically dominant spinocerebellar ataxias (SCAs). Ultimately, we suggest that SSBs might also be an etiological factor in normal human ageing. Finally, again based on new data, we propose that SSBs induce neurodegeneration by triggering over-activation of the SSB sensor protein, PARP1; thereby identifying inhibitors of this protein (currently licensed for cancer treatment) as a possible therapy for neurodegeneration. We will now address these hypotheses using a range of cutting edge molecular/cellular techniques. In particular we will (a), systematically examine all relevant amyotrophic lateral sclerosis/motor neurone disease (ALS/MND) and spinocerebellar ataxia (SCA) proteins for involvement in the DNA damage response, (b) Identify the mechanism/s by which ALS and SCA proteins engage in the DNA damage response, (c) Identify the role of ALS and SCA proteins in the DNA damage response, and (d) Explore PARP1 as a possible therapeutic target for treatment of neurodegenerative disease.


year authors and title journal last update
List of publications.
2019 Areej Mahjoub, Zuzana Cihlarova, Martine Tétreault, Lauren MacNeil, Neal Sondheimer, Keith W. Caldecott, Hana Hanzlikova, Grace Yoon
Homozygous pathogenic variant in BRAT1 associated with nonprogressive cerebellar ataxia
published pages: e359, ISSN: 2376-7839, DOI: 10.1212/nxg.0000000000000359
Neurology Genetics 5/5 2019-12-16
2018 Guido Zagnoli-Vieira, BSc, Francesco Bruni, PhD,* Kyle Thompson, PhD, Langping He, MD, PhD, Sarah Walker, PhD, Arjan P.M. de Brouwer, PhD, Robert Taylor, PhD, FRCPath, Dmitriy Niyazov, MD, and Keith W. Caldecott, PhD
Confirming TDP2 mutation in spinocerebellar ataxia autosomal recessive 23 (SCAR23)
published pages: e277, ISSN: 2376-7839, DOI: 10.1212/NXG.0000000000000277
Neurology Genetics 4/5 2019-09-04
2019 Maria Isabel Martinez-Macias, Duncan AQ Moore, Ryan L Green, Fernando Gomez-Herreros, Marcel Naumann, Andreas Hermann, Philip Van Damme, Majid Hafezparast, Keith W Caldecott
FUS (fused in sarcoma) is a component of the cellular response to topoisomerase I–induced DNA breakage and transcriptional stress
published pages: e201800222, ISSN: 2575-1077, DOI: 10.26508/lsa.201800222
Life Science Alliance 2/2 2019-09-04
2018 Hana Hanzlikova, Ilona Kalasova, Annie A. Demin, Lewis E. Pennicott, Zuzana Cihlarova, Keith W. Caldecott
The Importance of Poly(ADP-Ribose) Polymerase as a Sensor of Unligated Okazaki Fragments during DNA Replication
published pages: 319-331.e3, ISSN: 1097-2765, DOI: 10.1016/j.molcel.2018.06.004
Molecular Cell 71/2 2019-09-04

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