Fibrinogen storage disease without hypofibrinogenemia associated with estrogen therapy

BACKGROUND: Cytoplasmic inclusion bodies within hepatocytes may have different etiologies, including the Endoplasmic Reticulum Storage Diseases (ERSDs). ERSD is a pathological condition characterized by abnormal accumulation of proteins destined for secretion in the endoplasmic reticulum of hepatocytes; it may be congenital (primary) or acquired (secondary). Fibrinogen storage disease is a form of ERSD. CASE PRESENTATION: We present a case of fibrinogen storage disease secondary to estrogen replacement therapy. Its causal relationship to the drug is shown by histological, immunohistochemical and ultrastructural studies of paired liver biopsies obtained during and after the drug therapy. CONCLUSION: The liver biopsies of patients with idiopathic liver enzyme abnormalities should be carefully evaluated for cytoplasmic inclusion bodies and, although rare, fibrinogen deposits

Herschel/HIFI observations of interstellar OH+ and H2O+ towards W49N: a probe of diffuse clouds with a small molecular fraction

We report the detection of absorption by interstellar hydroxyl cations and water cations, along the sight-line to the bright continuum source W49N. We have used Herschel's HIFI instrument, in dual beam switch mode, to observe the 972 GHz N = 1 - 0 transition of OH+ and the 1115 GHz 1(11) - 0(00) transition of ortho-H2O+. The resultant spectra show absorption by ortho-H2O+, and strong absorption by OH+, in foreground material at velocities in the range 0 to 70 km/s with respect to the local standard of rest. The inferred OH+/H2O+ abundance ratio ranges from ~ 3 to ~ 15, implying that the observed OH+ arises in clouds of small molecular fraction, in the 2 - 8% range. This conclusion is confirmed by the distribution of OH+ and H2O+ in Doppler velocity space, which is similar to that of atomic hydrogen, as observed by means of 21 cm absorption measurements, and dissimilar from that typical of other molecular tracers. The observed OH+/H abundance ratio of a few E-8 suggests a cosmic ray ionization rate for atomic hydrogen of (0.6 - 2.4) E-16 s-1, in good agreement with estimates inferred previously for diffuse clouds in the Galactic disk from observations of interstellar H3+ and other species.Comment: Accepted for publication in A&A Letter

Energy Policy at Crossroad: potentials for sustainable energy transition in the Middle East and North African region

Countries of the Middle East and North African (MENA) region such as Morocco, Jordan and Tunisia, are facing challenges which require systems analysis. These challenges are connected with the growing energy demand and the need to diversify energy supply while addressing targets of climate change mitigation and energy security policies. At the same time the countries of the MENA region are also facing challenges of socio-economic development, such as the need in creation of jobs and multiplier effects for national economies as well as of further technological development and political transformation. Deployment of new and upgrading of existing electricity infrastructure, including generation, transmission and distribution systems, is an important prerequisite for sustainable development and economic growth. Energy policy solutions are needed for further upgrading of electricity system, which should be cost efficient, should support multiple development objectives and be based on compromise solutions involving a variety of views as well as perceptions of risks and benefits of various technologies from different stakeholders’ groups. The goal of this research was to explore economic, social, political and environmental effects on national and local levels of different electricity pathways for the period of up to the year 2050 in three countries of the MENA region. The methodology of this research was based on integrated and interdisciplinary approach while applying various methods of stakeholders’ dialogue such as multi criteria decision analysis, participatory modeling and others

Detection of interstellar oxidaniumyl: abundant H2O+ towards the star-forming regions DR21, Sgr B2, and NGC6334

We identify a prominent absorption feature at 1115 GHz, detected in first HIFI spectra towards high-mass star-forming regions, and interpret its astrophysical origin. The characteristic hyperfine pattern of the H2O+ ground-state rotational transition, and the lack of other known low-energy transitions in this frequency range, identifies the feature as H2O+ absorption against the dust continuum background and allows us to derive the velocity profile of the absorbing gas. By comparing this velocity profile with velocity profiles of other tracers in the DR21 star-forming region, we constrain the frequency of the transition and the conditions for its formation. In DR21, the velocity distribution of H2O+ matches that of the [CII] line at 158\mu\m and of OH cm-wave absorption, both stemming from the hot and dense clump surfaces facing the HII-region and dynamically affected by the blister outflow. Diffuse foreground gas dominates the absorption towards Sgr B2. The integrated intensity of the absorption line allows us to derive lower limits to the H2O+ column density of 7.2e12 cm^-2 in NGC 6334, 2.3e13 cm^-2 in DR21, and 1.1e15 cm^-2 in Sgr B2.Comment: Accepted for publication in A&

Modulation of cancer cell growth and progression by Caveolin-1 in the tumor microenvironment

Caveolin-1 (Cav-1), a major structural component of cell membrane caveolae, is involved in a variety of intracellular signaling pathways as well as transmembrane transport. Cav-1, as a scaffolding protein, modulates signal transduction associated with cell cycle progression, cellular senescence, cell proliferation and death, lipid homeostasis, etc. Cav-1 is also thought to regulate the expression or activity of oncoproteins, such as Src family kinases, H-Ras, protein kinase C, epidermal growth factor, extracellular signal-regulated kinase, and endothelial nitric oxide synthase. Because of its frequent overexpression or mutation in various tumor tissues and cancer cell lines, Cav-1 has been speculated to play a role as an oncoprotein in cancer development and progression. In contrast, Cav-1 may also function as a tumor suppressor, depending on the type of cancer cells and/or surrounding -stromal cells in the tumor microenvironment as well as the stage of tumors.