Mollusk freaks: New teratological cases on marine mollusks from the south pacific ocean

Indexación: Scopus.The present study provides new documented cases of abnormalities on chitons (hypomerism and coalescence of shell plates), in addition to four new cases on keyhole limpets (closed apical opening), and one new teratologic case on internal organs in octopuses (missing gill). We assess the frequency of these abnormalities and discuss about its possible environmental, mechanic and genetic causes. Several of these findings represent the first of these cases reported in South Pacific Ocean. © 2018, Escuela de Ciencias del Mar. All rights reserved.http://lajar.ucv.cl/index.php/rlajar/article/view/vol46-issue4-fulltext-

Cellular signaling in PKD: foreword

This monograph is dedicated to the memory of Dr. Jared James Grantham (1936–2016), a wonderful man, a compassionate physician, a passionate researcher, and an exceptional scientist. Without his vision, achievements and impact on countless collaborators and disciples, the field of Polycystic Kidney Disease would not be where it is today. His intellect, tenacity, modesty and kindness continue to be an inspiration to all

Fractional conservation laws in optimal control theory

Using the recent formulation of Noether's theorem for the problems of the calculus of variations with fractional derivatives, the Lagrange multiplier technique, and the fractional Euler-Lagrange equations, we prove a Noether-like theorem to the more general context of the fractional optimal control. As a corollary, it follows that in the fractional case the autonomous Hamiltonian does not define anymore a conservation law. Instead, it is proved that the fractional conservation law adds to the Hamiltonian a new term which depends on the fractional-order of differentiation, the generalized momentum, and the fractional derivative of the state variable.Comment: The original publication is available at http://www.springerlink.com Nonlinear Dynamic

Generation and phenotypic characterization of Pde1a mutant mice

Contains fulltext : 177029.pdf (publisher's version ) (Open Access)It has been proposed that a reduction in intracellular calcium causes an increase in intracellular cAMP and PKA activity through stimulation of calcium inhibitable adenylyl cyclase 6 and inhibition of phosphodiesterase 1 (PDE1), the main enzymes generating and degrading cAMP in the distal nephron and collecting duct, thus contributing to the development and progression of autosomal dominant polycystic kidney disease (ADPKD). In zebrafish pde1a depletion aggravates and overexpression ameliorates the cystic phenotype. To study the role of PDE1A in a mammalian system, we used a TALEN pair to Pde1a exon 7, targeting the histidine-aspartic acid dipeptide involved in ligating the active site Zn++ ion to generate two Pde1a null mouse lines. Pde1a mutants had a mild renal cystic disease and a urine concentrating defect (associated with upregulation of PDE4 activity and decreased protein kinase A dependent phosphorylation of aquaporin-2) on a wild-type genetic background and aggravated renal cystic disease on a Pkd2WS25/- background. Pde1a mutants additionally had lower aortic blood pressure and increased left ventricular (LV) ejection fraction, without a change in LV mass index, consistent with the high aortic and low cardiac expression of Pde1a in wild-type mice. These results support an important role of PDE1A in the renal pathogenesis of ADPKD and in the regulation of blood pressure

Radiative open charm decay of the Y(3940), Z(3930), X(4160) resonances

We determine the radiative decay amplitudes for decay into $D^*$ and $\bar{D} \gamma$, or $D^*_s$ and $\bar{D}_s \gamma$ of some of the charmonium like states classified as X,Y,Z resonances, plus some other hidden charm states which are dynamically generated from the interaction of vector mesons with charm. The mass distributions as a function of the $\bar{D} \gamma$ or $\bar{D}_s \gamma$ invariant mass show a peculiar behavior as a consequence of the $D^* \bar{D}^*$ nature of these states. The experimental search of these magnitudes can shed light on the nature of these states.Comment: 18 pages, 9 figure

Dynamically generated resonances from the vector octet-baryon decuplet interaction

We study the interaction of the octet of vector mesons with the decuplet of baryons using Lagrangians of the hidden gauge theory for vector interactions. The unitary amplitudes in coupled channels develop poles that can be associated with some known baryonic resonances, while there are predictions for new ones at the energy frontier of the experimental research. The work offers guidelines on how to search for these resonances

Recent experimental results in sub- and near-barrier heavy ion fusion reactions

Recent advances obtained in the field of near and sub-barrier heavy-ion fusion reactions are reviewed. Emphasis is given to the results obtained in the last decade, and focus will be mainly on the experimental work performed concerning the influence of transfer channels on fusion cross sections and the hindrance phenomenon far below the barrier. Indeed, early data of sub-barrier fusion taught us that cross sections may strongly depend on the low-energy collective modes of the colliding nuclei, and, possibly, on couplings to transfer channels. The coupled-channels (CC) model has been quite successful in the interpretation of the experimental evidences. Fusion barrier distributions often yield the fingerprint of the relevant coupled channels. Recent results obtained by using radioactive beams are reported. At deep sub-barrier energies, the slope of the excitation function in a semi-logarithmic plot keeps increasing in many cases and standard CC calculations over-predict the cross sections. This was named a hindrance phenomenon, and its physical origin is still a matter of debate. Recent theoretical developments suggest that this effect, at least partially, may be a consequence of the Pauli exclusion principle. The hindrance may have far-reaching consequences in astrophysics where fusion of light systems determines stellar evolution during the carbon and oxygen burning stages, and yields important information for exotic reactions that take place in the inner crust of accreting neutron stars.Comment: 40 pages, 63 figures, review paper accepted for EPJ

Confirmation of the Double Charm Baryon Xi_cc+ via its Decay to p D+ K-

We observes a signal for the double charm baryon Xi_cc+ in the charged decay mode Xi_cc+ -> p D+ K- to complement the previously reported decay Xi_cc+ -> Lambda_c K- pi+ in data from SELEX, the charm hadro-production experiment (E781) at Fermilab. In this new decay mode we observe an excess of 5.62 events over an expected background estimated by event mixing to be 1.38+/-0.13 events. The Poisson probability that a background fluctuation can produce the apparent signal is less than 6.4E-4. The observed mass of this state is (3518+/-3)MeV/c^2, consistent with the published result. Averaging the two results gives a mass of (3518.7+/-1.7)MeV/c^2. The observation of this new weak decay mode confirms the previous SELEX suggestion that this state is a double charm baryon. The relative branching ratio Gamma(Xi_cc+ -> pD+K-)/Gamma(Xi_cc+ -> Lambda_c K- pi+) = 0.36+/-0.21.Comment: 11 pages, 6 included eps figures. v2 includes improved statistical method to determine significance of observation. Submitted to PL

Venglustat, a novel glucosylceramide synthase inhibitor, in patients at risk of rapidly progressing ADPKD: primary results of a double-blind, placebo-controlled, phase 2/3 randomized clinical trial

RATIONALE AND OBJECTIVE: Autosomal dominant polycystic kidney disease (ADPKD) is characterized by the formation of multiple kidney cysts that leads to growth in total kidney volume (TKV) and progression to kidney failure. Venglustat is a glucosylceramide synthase inhibitor that has been shown to inhibit cyst growth and reduce kidney failure in preclinical models of ADPKD. STUDY DESIGN: STAGED-PKD was a two-stage, multicenter, double-blind, randomized, placebo-controlled Phase 2/3 study in adults with ADPKD at risk of rapidly progressive disease, selected based on Mayo Kidney Volume Class 1C-1E and an estimated glomerular filtration rate (eGFR) of 30-89.9 mL/min/1.73 m2. SETTING AND PARTICIPANTS: Enrollment included 236 and 242 patients in Stages 1 and 2, respectively. INTERVENTION(S): In Stage 1, patients were randomized 1:1:1 to venglustat 8 mg or 15 mg, or placebo. In Stage 2, patients were randomized 1:1 to venglustat 15 mg (highest dose identified as safe and well tolerated in Stage 1) or placebo. OUTCOMES: Primary endpoints were rate of change in TKV over 18 months in Stage 1 and eGFR slope over 24 months in Stage 2. Secondary endpoints were eGFR slope over 18 months (Stage 1), rate of change in TKV (Stage 2), and safety/tolerability, pain, and fatigue (Stages 1 and 2). RESULTS: A prespecified interim futility analysis showed that venglustat treatment had no effect on the annualized rate of change in TKV over 18 months (Stage 1) and had a faster rate of decline in eGFR slope over 24 months (Stage 2). Due to this lack of efficacy, the study was terminated early. LIMITATIONS: The short follow-up after end-of-treatment and limited generalizability of findings. CONCLUSIONS: In patients with rapidly progressing ADPKD, treatment with venglustat at either 8 mg or 15 mg showed no change in the rate of change in TKV and a faster rate of eGFR decline in the STAGED-PKD trial despite a dose-dependent decrease in plasma glucosylceramide (GL-1) levels