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SIDSCA SIGNED

Defective DNA Damage Responses in Dominant Neurodegenerative Diseases

Total Cost €

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EC-Contrib. €

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Partnership

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 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.

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

Project "SIDSCA" data sheet

The following table provides information about the project.

Coordinator
THE UNIVERSITY OF SUSSEX 

Organization address
address: SUSSEX HOUSE FALMER
city: BRIGHTON
postcode: BN1 9RH
website: http://www.sussex.ac.uk

contact info
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 Coordinator Country United Kingdom [UK]
 Project website http://www.sussex.ac.uk/lifesci/caldecottlab/
 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

 Partnership

Take a look of project's partnership.

# participants  country  role  EC contrib. [€] 
1    THE UNIVERSITY OF SUSSEX UK (BRIGHTON) coordinator 1˙783˙034.00
2    USTAV MOLEKULARNI GENETIKY AKADEMIE VED CESKE REPUBLIKY VEREJNA VYZKUMNA INSTITUCE CZ (PRAHA 4) participant 664˙375.00

Map

 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.

 Publications

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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