This report concerns a boy presenting renal disease with tubulointerstitial nephropathy which suggests familial juvenile nephronophthisis, hepatosplenomegaly due to congenital hepatic fibrosis, ...tapetoretinal degeneration, probable endocrine involvement and congenital skeletal abnormalities. The associations presented in this paper have not previously been reported.
Omega Centauri is no longer the only globular cluster known to contain multiple stellar populations, yet it remains the most puzzling. Due to the extreme way in which the multiple stellar population ...phenomenon manifests in this cluster, it has been suggested that it may be the remnant of a larger stellar system. In this work, we present a spectroscopic investigation of the stellar populations hosted in the globular cluster Delta *w Centauri to shed light on its still puzzling chemical enrichment history. With this aim, we used FLAMES+GIRAFFEatVLT to observe 300 stars distributed along the multimodal red giant branch of this cluster, sampling with good statistics the stellar populations of different metallicities. We determined chemical abundances for Fe, Na, O, and n-capture elements Ba and La. We confirm that Delta *w Centauri exhibits large star-to-star variations in iron with Fe/H ranging from ~--2.0 to ~--0.7 dex. Barium and lanthanum abundances of metal-poor stars are correlated with iron, up to Fe/H ~--1.5, while they are almost constant (or at least have only a moderate increase) in the more metal-rich populations. There is an extended Na-O anticorrelation for stars with Fe/H --1.3 while more metal-rich stars are almost all Na-rich. Sodium was found to mildly increase with iron over the entire metallicity range.
Poly(ADP-ribose) polymerase (PARP) inhibitors represent a promising strategy toward the treatment of triple-negative breast cancer (TNBC), which is often associated to genomic instability and/or BRCA ...mutations. However, clinical outcome is controversial and no benefits have been demonstrated in wild type BRCA cancers, possibly due to poor drug bioavailability and low nuclear delivery. In the attempt to overcome these limitations, we have developed H-Ferritin nanoformulated olaparib (HOla) and assessed its anticancer efficacy on both BRCA-mutated and non-mutated TNBC cells. We exploited the natural tumor targeting of H-Ferritin, which is mediated by the transferrin receptor-1 (TfR1), and its physiological tropism toward cell nucleus. TNBC cell lines over-expressing TfR-1 were successfully recognized by H-Ferritin, displaying a fast internalization into the cells. HOla induced remarkable cytotoxic effect in cancer cells, exhibiting 1000-fold higher anticancer activity compared to free olaparib (Ola). Accordingly, HOla treatment enhanced PARP-1 cleavage, DNA double strand breaks and Ola delivery into the nuclear compartment. Our findings suggest that H-Ferritin nanoformulation strongly enhances cytotoxic efficacy of Ola as a stand-alone therapy in both BRCA-mutated and wild type TNBC cells, by promoting targeted nuclear delivery.
Generalized roughness is the most common damage occurring to rolling bearings. It produces a frequency spreading of the characteristic fault frequencies, thus making it difficult to detect with ...spectral or envelope analysis. A statistical analysis of typical bearing faults is proposed here in order to identify the spreading bandwidth related to specific conditions, relying on current or vibration measurements only. Then, a diagnostic index based on the computation of the energy in the previously defined bandwidth is used to diagnose bearing faults. The proposed method was validated experimentally with vibration signals, with robust and reliable results. The same procedure can be extended to current signals.
Thoracic aortic aneurysms are life-threatening lesions that afflict young and old individuals alike. They frequently associate with genetic mutations and are characterized by reduced elastic fibre ...integrity, dysfunctional smooth muscle cells, improperly remodelled collagen and pooled mucoid material. There is a pressing need to understand better the compromised structural integrity of the aorta that results from these genetic mutations and renders the wall vulnerable to dilatation, dissection or rupture. In this paper, we compare the biaxial mechanical properties of the ascending aorta from 10 murine models: wild-type controls, acute elastase-treated, and eight models with genetic mutations affecting extracellular matrix proteins, transmembrane receptors, cytoskeletal proteins, or intracellular signalling molecules. Collectively, our data for these diverse mouse models suggest that reduced mechanical functionality, as indicated by a decreased elastic energy storage capability or reduced distensibility, does not predispose to aneurysms. Rather, despite normal or lower than normal circumferential and axial wall stresses, it appears that intramural cells in the ascending aorta of mice prone to aneurysms are unable to maintain or restore the intrinsic circumferential material stiffness, which may render the wall biomechanically vulnerable to continued dilatation and possible rupture. This finding is consistent with an underlying dysfunctional mechanosensing or mechanoregulation of the extracellular matrix, which normally endows the wall with both appropriate compliance and sufficient strength.
Multi-band Hubble Space Telescope photometry reveals that the main sequence, sub-giant, and the red-giant branch of the globular cluster NGC 6752 splits into three main components in close analogy ...with the three distinct segments along its horizontal branch stars. These triple sequences are consistent with three stellar groups: a stellar population with a chemical composition similar to field-halo stars (Population a), a Population (c) with enhanced sodium and nitrogen, depleted carbon and oxygen, and an enhanced helium abundance ( Delta Y ~ 0.03), and a Population (b) with an intermediate (between Populations a and c) chemical composition and slightly enhanced helium ( Delta Y ~ 0.01). These components contain ~25% (Population a), ~45% (Population b), and ~30% (Population c) of the stars. No radial gradient for the relative numbers of the three populations has been identified out to about 2.5 half-mass radii.
In this paper, we combine Wide Field Camera3/UVIS F275W, F336W, and F438W data from the ‘UV Legacy Survey of Galactic Globular Clusters: Shedding Light on Their Populations and Formation’ (GO 13297) ...Hubble Space Telescope Treasury programme with F606W, F625W, F658N, and F814W Advanced Camera for Surveys archive data for a multiwavelength study of the globular cluster NGC 6352. In the colour–magnitude and two-colour diagrams obtained with appropriate combination of the photometry in the different bands, we separate two distinct stellar populations and trace them from the main sequence to the subgiant, red giant, horizontal and asymptotic giant branches. We infer that the two populations differ in He by ΔY = 0.029 ± 0.006. With a new method, we also estimate the age difference between the two sequences. Assuming no difference in Fe/H and α/Fe, and the uncertainties on ΔY, we found a difference in age between the two populations of 10 ± 120 Myr. If we assume Fe/H and α/Fe differences of 0.02 dex (well within the uncertainties of spectroscopic measurements), the total uncertainty in the relative age rises to ∼300 Myr.
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