Cultural Heritage Image Sharing Recommendations Report

Deliverable 13.2 for the WorldFAIR Project’s Cultural Heritage Work Package (WP13). Although the cultural heritage sector has only recently begun to think of traditional gallery, library, archival and museum (‘GLAM’) collections as data, long established practices guiding the management and sharing of information resources has aligned the domain well with the FAIR principles for research data, evidenced in complementary workflows and standards that support discovery, access, reuse, and persistence. As explored in the previous report by Work Package 13 for the WorldFAIR Project, D13.1 Practices and policies supporting cultural heritage image sharing platforms, memory institutions are in an important position to influence cross-domain data sharing practices and raise critical questions about why and how those practices are implemented. Deliverable 13.2 aims to build on our understanding of what it means to support FAIR in the sharing of image data derived from GLAM collections. This report looks at previous efforts by the sector towards FAIR alignment and presents 5 recommendations designed to be implemented and tested at the DRI that are also broadly applicable to the work of the GLAMs. The recommendations are ultimately a roadmap for the Digital Repository of Ireland (DRI) to follow in improving repository services, as well as a call for continued dialogue around ‘what is FAIR?’ within the cultural heritage research data landscape. Visit WorldFAIR online at http://worldfair-project.eu. WorldFAIR is funded by the EC HORIZON-WIDERA-2021-ERA-01-41 Coordination and Support Action under Grant Agreement No. 101058393

Mechanisms of disease in frontotemporal lobar degeneration: gain of function versus loss of function effects

Frontotemporal lobar degeneration (FTLD) is clinically, pathologically and genetically heterogeneous. The prototypical clinical syndromes are behavioural variant frontotemporal dementia (bvFTD), a disorder of behaviour and executive impairments, progressive non-fluent aphasia (PNFA), a disorder of expressive language, and semantic dementia (SD), a disorder of conceptual knowledge [Neary et al 1998]. A proportion of patients with any of these syndromes of FTLD can develop the amyotrophic form of motor neurone disease (MND) [Neary et al 1990, Strong et al], further emphasising clinical heterogeneity within FTLD, and highlighting the long known association with, and suspected pathogenetic links between, FTLD and MND