6 research outputs found

    Building Community with Diversity, Equity and Inclusion Principles

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    DEI has become increasingly important for all libraries because while the populations we serve are more diverse our staff is not. What are initiatives that I can do to support an inclusive culture for the library, campus and profession?https://digitalworks.union.edu/diversityinclusioncourse_posters_2018/1001/thumbnail.jp

    Voices in the Library

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    In early 2012, a team of librarians from Emory University\u27s Robert W Woodruff Library set out to better understand how the library\u27s key user groups work, what they need to do that work, and how the library might better meet those needs. To develop its understanding the team modified the Voice of the Customer FOCUS methodology--a clearly defined and rigorous process for developing and conducting semi-structured interviews and for analyzing the data associated with those interviews. In this presentation, team members will review their research methodology (including a discussion of its benefits and drawbacks), review their project\u27s findings and its impact on the library, and suggest how others might adapt their research methodology to their own libraries and needs

    Impaired antibacterial autophagy links granulomatous intestinal inflammation in Niemann–Pick disease type C1 and XIAP deficiency with NOD2 variants in Crohn's disease

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    Objective Patients with Niemann–Pick disease type C1 (NPC1), a lysosomal lipid storage disorder that causes neurodegeneration and liver damage, can present with IBD, but neither the significance nor the functional mechanism of this association is clear. We studied bacterial handling and antibacterial autophagy in patients with NPC1. Design We characterised intestinal inflammation in 14 patients with NPC1 who developed IBD. We investigated bacterial handling and cytokine production of NPC1 monocytes or macrophages in vitro and compared NPC1-associated functional defects to those caused by IBD-associated nucleotide-binding oligomerization domain-containing protein 2 (NOD2) variants or mutations in X-linked inhibitor of apoptosis (XIAP). Results Patients with the lysosomal lipid storage disorder NPC1 have increased susceptibility to early onset fistulising colitis with granuloma formation, reminiscent of Crohn’s disease (CD). Mutations in NPC1 cause impaired autophagy due to defective autophagosome function that abolishes NOD2-mediated bacterial handling in vitro similar to variants in NOD2 or XIAP deficiency. In contrast to genetic NOD2 and XIAP variants, NPC1 mutations do not impair NOD2-receptorinteracting kinase 2 (RIPK2)-XIAP-dependent cytokine production. Pharmacological activation of autophagy can rescue bacterial clearance in macrophages in vitro by increasing the autophagic flux and bypassing defects in NPC1. Conclusions NPC1 confers increased risk of earlyonset severe CD. Our data support the concept that genetic defects at different checkpoints of selective autophagy cause a shared outcome of CD-like immunopathology linking monogenic and polygenic forms of IBD. Muramyl dipeptide-driven cytokine responses and antibacterial autophagy induction are parallel and independent signalling cascades downstream of the NOD2-RIPK2-XIAP complex
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