#	Pagina
attuale pagina	/open-h2020/projects/204857/index.html
-1	/open-h2020/projects/214381/index.html
-2	/open-h2020/projects/221917/index.html
-3	/open-h2020/per-topic/ibf/list/index.html
-4	/open-h2020/per-topic/fede/list/index.html

Opendata, web and dolomites

SIDSCA SIGNED

Defective DNA Damage Responses in Dominant Neurodegenerative Diseases

Total Cost €

EC-Contrib. €

Partnership

Views

Outcomes and
results

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.

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

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 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	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 THE UNIVERSITY OF SUSSEX Organization address address: SUSSEX HOUSE FALMER city: BRIGHTON postcode: BN1 9RH website: http://www.sussex.ac.uk contact info title: n.a. name: n.a. surname: n.a. function: n.a. email: n.a. telephone: n.a. fax: n.a.	UK (BRIGHTON)	coordinator	1˙783˙034.00
2	USTAV MOLEKULARNI GENETIKY AKADEMIE VED CESKE REPUBLIKY VEREJNA VYZKUMNA INSTITUCE USTAV MOLEKULARNI GENETIKY AKADEMIE VED CESKE REPUBLIKY VEREJNA VYZKUMNA INSTITUCE Organization address address: VIDENSKA 1083 city: PRAHA 4 postcode: 142 20 website: www.img.cas.cz contact info title: n.a. name: n.a. surname: n.a. function: n.a. email: n.a. telephone: n.a. fax: n.a.	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

List of publications.
year	authors and title	journal	last update
2019	Areej Mahjoub, Zuzana Cihlarova, Martine TÃ©treault, 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 Iâ€“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

Are you the coordinator (or a participant) of this project? Plaese send me more information about the "SIDSCA" project.

For instance: the website url (it has not provided by EU-opendata yet), the logo, a more detailed description of the project (in plain text as a rtf file or a word file), some pictures (as picture files, not embedded into any word file), twitter account, linkedin page, etc.

Send me an email (fabio@fabiodisconzi.com) and I put them in your project's page as son as possible.

Thanks. And then put a link of this page into your project's website.

The information about "SIDSCA" are provided by the European Opendata Portal: CORDIS opendata.