A Collaboration Service Model for a Global Port Cluster

The importance of port clusters to a global city may be viewed from a number of perspectives. The development of port clusters and economies of agglomeration and their contribution to a regional economy is underpinned by information and physical infrastructure that facilitates collaboration between business entities within the cluster. The maturity of technologies providing portals, web and middleware services provides an opportunity to push the boundaries of contemporary service reference models and service catalogues to what the authors propose to be “collaboration services”. Servicing port clusters, portal engineers of the future must consider collaboration services to benefit a region. Particularly, service orchestration through a “public user portal” must gain better utilisation of publically owned infrastructure, to share knowledge and collaborate among organisations through information systems

Karyn Welsh (Innovation and Ideas Series)

We are pleased to have our Supply Chain Innovation Industry Partner Karyn Welsh, CEO of the CILT Australia present next up in our Innovation and Ideas series. Karyn discusses the innovation coming out of the supply chain industry after the impact of COVID-19 which pushed an industry that was a lager in the adoption of technology into the digital world at such a fast pace where everyone has embraced the technology but not necessarily spent a lot of time thinking through that technology

Exploring the relevance of NUP93 variants in steroid-resistant nephrotic syndrome using next generation sequencing and a fly kidney model.

BACKGROUND: Variants in genes encoding nuclear pore complex (NPC) proteins are a newly identified cause of paediatric steroid-resistant nephrotic syndrome (SRNS). Recent reports describing NUP93 variants suggest these could be a significant cause of paediatric onset SRNS. We report NUP93 cases in the UK and demonstrate in vivo functional effects of Nup93 depletion in a fly (Drosophila melanogaster) nephrocyte model. METHODS: Three hundred thirty-seven paediatric SRNS patients from the National cohort of patients with Nephrotic Syndrome (NephroS) were whole exome and/or whole genome sequenced. Patients were screened for over 70 genes known to be associated with Nephrotic Syndrome (NS). D. melanogaster Nup93 knockdown was achieved by RNA interference using nephrocyte-restricted drivers. RESULTS: Six novel homozygous and compound heterozygous NUP93 variants were detected in 3 sporadic and 2 familial paediatric onset SRNS characterised histologically by focal segmental glomerulosclerosis (FSGS) and progressing to kidney failure by 12 months from clinical diagnosis. Silencing of the two orthologs of human NUP93 expressed in D. melanogaster, Nup93-1, and Nup93-2 resulted in significant signal reduction of up to 82% in adult pericardial nephrocytes with concomitant disruption of NPC protein expression. Additionally, nephrocyte morphology was highly abnormal in Nup93-1 and Nup93-2 silenced flies surviving to adulthood. CONCLUSION: We expand the spectrum of NUP93 variants detected in paediatric onset SRNS and demonstrate its incidence within a national cohort. Silencing of either D. melanogaster Nup93 ortholog caused a severe nephrocyte phenotype, signaling an important role for the nucleoporin complex in podocyte biology. A higher resolution version of the Graphical abstract is available as Supplementary information