Distribución temporal del tamaño de los molariformes de Ursus spelaeus Ros.-Hein.ibérico

[Abstract] This paper deals with a metrical comparison of cheek-teeth length of Ursus spelaeus Ros.-Hein.Iberian population representatives

Detection of an optical transient following the 13 March 2000 short/hard gamma-ray burst

We imaged the error box of a gamma-ray burst of the short (0.5 s), hard type (GRB 000313), with the BOOTES-1 experiment in southern Spain, starting 4 min after the gamma-ray event, in the I-band. A bright optical transient (OT 000313) with I = 9.4 +/- 0.1 was found in the BOOTES-1 image, close to the error box (3-sigma) provided by BATSE. Late time VRIK'-band deep observations failed to reveal an underlying host galaxy. If the OT 000313 is related to the short, hard GRB 000313, this would be the first optical counterpart ever found for this kind of events (all counterparts to date have been found for bursts of the long, soft type). The fact that only prompt optical emission has been detected (but no afterglow emission at all, as supported by theoretical models) might explain why no optical counterparts have ever been found for short, hard GRBs.This fact suggests that most short bursts might occur in a low-density medium and favours the models that relate them to binary mergers in very low-density enviroments.Comment: Revised version. Accepted for publication in Astronomy and Astrophysics Letters, 5 pages, 3 figure

The FXII c.-4T > C Polymorphism as a Disease Modifier in Patients With Hereditary Angioedema Due to the FXII p.Thr328Lys Variant

Background: Hereditary angioedema due to the Thr328Lys variant in the coagulation factor XII (HAE-FXII) affects mainly women in whom the symptomatology is dependent on high estrogen levels. Clinical variability and incomplete penetrance are challenging features that hinder the diagnosis and management of HAE-FXII. The c.-4T>C Kozak polymorphism is the only common variation accounting for FXII plasma levels and was previously shown to modify the course of HAE due to C1-Inhibitor deficiency. Objectives: To assess the influence of the c.-4T>C polymorphism on disease expression in 39 Spanish HAE-FXII index patients. Methods: The c.-4T>C polymorphism was sequenced by the standard Sanger method, and HAE severity was calculated according to the score by Cumming et al. (2003) The activation of the contact system was quantified by the kallikrein-like activity of plasma in chromogenic assays upon activation with high-molecular-weight dextran sulfate. Results: The c.-4CC genotype was overrepresented in the studied cohort: 82% were CC-homozygous (expected frequency = 59%) and 18% were CT-heterozygous (expected frequency = 39%) (p = 0.001). Patients with a c.-4CC genotype exhibited higher kallikrein-like activity (0.9659 +/- 0.1136) than those with a c.-4TC genotype (0.7645 +/- 0.1235) (p = 0.024) or healthy donors. Moreover, the polymorphism influenced HAE-FXII severity score (c.-4CC = 4.43 +/- 2.28 vs c.-4TC = 2.0 +/- 1.15; p = 0.006) but not the degree of estrogen dependence or time until remission. Conclusion: The c.-4T>C polymorphism is overrepresented in a Spanish HAE-FXII cohort and significantly influences the degree of contact system activation and the clinical severity of the disease

The synaptic ribbon is critical for sound encoding at high rates and with temporal precision.

We studied the role of the synaptic ribbon for sound encoding at the synapses between inner hair cells (IHCs) and spiral ganglion neurons (SGNs) in mice lacking RIBEYE (RBEKO/KO). Electron and immunofluorescence microscopy revealed a lack of synaptic ribbons and an assembly of several small active zones (AZs) at each synaptic contact. Spontaneous and sound-evoked firing rates of SGNs and their compound action potential were reduced, indicating impaired transmission at ribbonless IHC-SGN synapses. The temporal precision of sound encoding was impaired and the recovery of SGN-firing from adaptation indicated slowed synaptic vesicle (SV) replenishment. Activation of Ca2+-channels was shifted to more depolarized potentials and exocytosis was reduced for weak depolarizations. Presynaptic Ca2+-signals showed a broader spread, compatible with the altered Ca2+-channel clustering observed by super-resolution immunofluorescence microscopy. We postulate that RIBEYE disruption is partially compensated by multi-AZ organization. The remaining synaptic deficit indicates ribbon function in SV-replenishment and Ca2+-channel regulation

Simultaneous and optical follow-up GRB observations by BOOTES

Since 1998 BOOTES has provided follow-up observations for more than 70 GRBs; the most important results obtained so far are the detection of an OT in the GRB 000313 error box and the non-detection of optical emission simultaneous to the high-energy emission for several GRBs (both long/soft and short/hard events)

Serum amyloid A primes microglia for ATP-dependent interleukin-1\u3b2 release

Acute-phase response is a systemic reaction to environmental/inflammatory insults and involves production of acute-phase proteins, including serum amyloid A (SAA). Interleukin-1\u3b2 (IL-1\u3b2), a master regulator of neuroinflammation produced by activated inflammatory cells of the myeloid lineage, in particular microglia, plays a key role in the pathogenesis of acute and chronic diseases of the peripheral nervous system and CNS. IL-1\u3b2 release is promoted by ATP acting at the purinergic P2X7 receptor (P2X7R) in cells primed with toll-like receptor (TLR) ligands

Parallax in “Pi of the Sky” project

The main goal of the “Pi of the Sky” project is search for optical transients (OTs) of astrophysical origin, in particular those related to gamma-ray bursts (GRBs). Since March 2011 the project has two running observatories: one in northern Chile and the other one insouthern Spain. This allows for regular observations of a common sky fields, visible from both observatories which are scheduled usually 1–2 h per night. In such a case, the on-line flash recognition algorithm, looking for optical transients, can use parallax information toassure that events observed from both sites have parallax angle smaller than the error of astrometry. On the other hand, the remaining OT candidates can be verified against a hypothesis of being near-Earth objects. This paper presents algorithm using parallax information for identification of near-Earth objects, which might be satellites, or space debris elements. Preliminary results of the algorithm are also presented

Hematopoietic Cell Transplantation in Patients With Primary Immune Regulatory Disorders (PIRD): A Primary Immune Deficiency Treatment Consortium (PIDTC) Survey.

Primary Immune Regulatory Disorders (PIRD) are an expanding group of diseases caused by gene defects in several different immune pathways, such as regulatory T cell function. Patients with PIRD develop clinical manifestations associated with diminished and exaggerated immune responses. Management of these patients is complicated; oftentimes immunosuppressive therapies are insufficient, and patients may require hematopoietic cell transplant (HCT) for treatment. Analysis of HCT data in PIRD patients have previously focused on a single gene defect. This study surveyed transplanted patients with a phenotypic clinical picture consistent with PIRD treated in 33 Primary Immune Deficiency Treatment Consortium centers and European centers. Our data showed that PIRD patients often had immunodeficient and autoimmune features affecting multiple organ systems. Transplantation resulted in resolution of disease manifestations in more than half of the patients with an overall 5-years survival of 67%. This study, the first to encompass disorders across the PIRD spectrum, highlights the need for further research in PIRD management