The essential oils extracted from mandarin (Citrus reticulata Blanco) fruits, and from tea tree (Maleleuca alternifolia (Maiden and Betche) Cheel) leaves have been chemically analyzed and tested for their bioactivity against D. suzukii. Besides, to estimate consumers’ acceptability of the essential oil (EO) treatments, we evaluated their impact on the organoleptic characteristics of the EO-treated fruits. The main chemical constituents of the two EOs were 1,8-cineole and 4-terpineol for M. alternifolia (22.4% and 17.6% of the total components, respectively), and limonene (83.6% of the total components) for C. reticulata. The behavioral tests indicate that the two EOs are able to deter D. suzukii oviposition and that D. suzukii shows positive chemotaxis to low concentrations of the EOs and negative chemotaxis when the EO concentration increases. While no negative effects on the organoleptic profiles were detected for fruits treated with C. reticulata EO, the olfactory profile of fruits treated with M. alternifolia EO was so negative that they were defined as “not suitable for consumption” by panellists. Overall, our findings indicate that the use of EOs for the post-harvest protection of small fruits is feasible, provided that the essential oils are selected not only for their bioactivity against the insect pest but also for their affinity with the consumers’ sensorial system.

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Axillary meristems (AMs) contribute to the growth of a plant, determining adult architecture and reproductive success in response to environmental stimuli. The missing flowers (mf ) mutant of sunflower (Helianthus annuus) is defective in AM development. mf lacks shoot branching and ray flowers, occasionally producing few disk flowers. Here we demonstrated that a point mutation in the REGULATOR OF AXILLARY MERISTEM FORMATION-LIKE (Ha-ROXL) gene of mf generates a premature stop codon and therefore a nonfunctional bHLH transcription factor, no longer localized in the nucleus, where it should exert its function. Virus-induced gene silencing of Ha-ROXL also causes defects in disk and ray flower development. Ha-ROXL mRNA accumulates at the adaxial boundaries of leaves and AMs. During inflorescence development, Ha-ROXL is expressed in small arcs of cells before a clear separation between abaxial bracts and disk flower primordia. No Ha-ROXL mRNA accumulates in mf inflorescences. Several genes known to play roles in plant architecture, auxin transport, and flower development are differentially expressed in mf and Ha-ROXL-silenced plants. These results highlight the predominant role of Ha-ROXL in regulating AMs in sunflower. In dicot, mf is the first mutant for which the ROXL gene is also required for initiation of flower meristems. K E Y W O R D S axillary meristems, bHLH transcription factor, floral meristems, Ha-ROXL, Helianthus annuus, in situ hybridization, missing flowers mutant, virus-induced gene silencing

Objectives. The purpose of this study was to review the frequency and clinical presentation of the rarest clinical manifestations of systemic lupus erythematosus (SLE).Methods. A list of 6 rare SLE manifestations were defined: gastrointestinal, liver, pulmonary, cardiac, ocular and neurological manifestations. Each topic was assigned to a couple of authors to perform a literature search and article review. Results. In total, 149 articles were included in the literature review: 37 for gastrointestinal manifestations, 6 for liver manifestations, 27 for pulmonary manifestations, 50 for cardiac manifestations, 16 for ocular manifestations, 13 for neurological manifestations. Gastrointestinal disorders included several clinical presentations with variable frequency (from 0.5% to 10.7% of the cases); liver involvement included lupusrelated hepatitis (9.3%) and autoimmune hepatitis (2.3%). The rarest pulmonary manifestations identified were shrinking lung syndrome, described in 1.5% of patients, while interstitial lung disease and lupus pneumonia were reported in 4% and 3% of patients, respectively. Myocarditis and pulmonary hypertension were also rarely described in SLE patients although ranging from 0.4-16% and 1-14% respectively, depending on the methodology used for its identification. Ocular manifestations in SLE included some rare manifestations (reported in less than 5% of patients) and lupus retinopathy that is described in 1.2-28.8% of patients depending on methods of ascertainment. Aseptic meningitis and chorea were also confirmed as very rare manifestations being reported in less than 1% and in 0.3-2.4% of cases respectively. Conclusion. The results of this literature review provide the basis for a better understanding of some less-known manifestations of SLE and for stressing the need for a higher awareness in diagnostic and therapeutic protocols regarding these rare disease aspects.

In order to address the main challenges related to the rare diseases (RDs) the European Commission launched the European Reference Networks (ERNs), virtual networks involving healthcare providers (HCPs) across Europe. The mission of the ERNs is to tackle low prevalence and RDs that require highly specialised treatment and a concentration of knowledge and resources. In fact, ERNs offer the potential to give patients and healthcare professionals across the EU access to the best expertise and timely exchange of lifesaving knowledge, trying to make the knowledge travelling more than patients. For this reason, ERNs were established as concrete European infrastructures, and this is particularly crucial in the framework of rare and complex diseases in which no country alone has the whole knowledge and capacity to treat all types of patients. It has been five years since their kick-off launch in Vilnius in 2017. The 24 ERNs have been intensively working on different transversal areas, including patient management, education, clinical practice guidelines, patients' care pathways and many other fundamental topics. The present work is therefore aimed not only at reporting a summary of the main activities and milestones reached so far, but also at celebrating the first 5 years of the ERN on Rare and Complex Connective Tissue and Musculo-skeletal Diseases (ReCONNET), in which the members of the network built together one of the 24 infrastructures that are hopefully going to change the scenario of rare diseases across the EU.