Activation of Stress Kinases in the Brain of Mucopolysaccharidosis IIIB mice

The accumulation of heparan sulfate (HS) in lysosomes is the primary consequence of the enzyme defect (α-N-acetylglucosaminidase) in mucopolysaccharidosis type IIIB. This accumulation triggers a cascade of pathological events that progressively leads to CNS pathology. Here we examined the activation of the three major stress kinases in the neuronal tissue of a murine model of the disease. ERK1/2 was significantly higher in the cortex of 1–2-month-old affected animals compared with wild-type (Wt) littermates. Similarly, ERK1/2 was stimulated in neurons cultured from MPS IIIB mice. SAPK/JNK was also found to be activated in the cortex of 1–2-month-old affected animals compared with Wt subjects, and the same was found for cultured neurons. In contrast, the active form of p38MAPK was lower in the cortex of 1-month-old MPS IIIB mice compared with Wt animals, but no significant difference was found between the two p38MAPK analyzed in normal and affected neurons cultured in vitro. These data indicate the possible involvement of MAPK dysregulation in the early stage of MPS IIIB brain disease. © 2011 Wiley-Liss, Inc

Anti-VEGF Therapy in Breast and Lung Mouse Models of Cancers

Cancer is the second leading cause of death in the world after cardiovascular diseases. Some types of cancer cells often travel to other parts of the body through blood circulation or lymph vessels, where they begin to grow. This process is recognized as metastasis. Angiogenesis is the formation of new blood vessels from existing vessel. Normally angiogenesis is a healthy process, that helps the body to heal wounds and repair damaged body tissues, whereas in cancerous condition this process supports new blood vessels formation that provide a tumor with its own blood supply, nutrients and allow it to grow. The most important proximal factor for angiogenesis is the vascular endothelial growth factor VEGF. Angioinhibition is a form of targeted therapy that uses drugs to stop tumors from making new blood vessels. Therefore, in this paper we analyse the importance of VEGF as target of cancer therapy, analysing murine models

Late diagnosis of Fabry disease caused by a de novo mutation in a patient with end stage renal disease.

BACKGROUND: We present the case of a white 35-year-old male with a diagnosis of Fabry disease and negative family history. CASE PRESENTATION: At the age of 31, he underwent a renal biopsy with a diagnosis of hypertension-induced nephroangiosclerosis. At the age of 35, he was referred to our hospital and started dialysis: the unusual finding of left ventricular hypertrophy with a normal ejection fraction and of myocardial fibrosis at the cardiac magnetic resonance suggested a diagnosis of Fabry disease, although there was no apparent family history—so extensive tests were subsequently undertaken. The patient had low plasma levels of α-galactosidase A and the genetic analysis showed a single nucleotide point mutation in hemizygosis at nucleotide c.901 C>T in exon 6 of the GLA gene, confirming the diagnosis of Fabry disease. We extended the genetic analysis to all family members of the patient (mother, sister and brothers) and none of them had any alteration in the GLA gene, suggesting a de novo mutation in the patient. CONCLUSIONS: In a family, it is rare to find only one Fabry disease affected subject with a de novo mutation. These findings emphasize the importance of early diagnosis, genetic counseling and studying the genealogical tree of suspicious patients, even in absence of a typical family history

Finding aquaporins in annelids : An evolutionary analysis and a case study

Funding Information: This research was funded by PRIN2017 Programmi di Ricerca Scientifica di Rilevante Interesse Nazionale 2017 (grant # 2017J92TM5) by Italian MUR to G.C. and by the Molecular Bio-diversity Laboratory of the Italian node of Lifewatch (CNR, Consiglio Nazionale delle Ricerche) to C.G.Peer reviewedPublisher PD

Correction to: A formulation of neem and hypericum oily extract for the treatment of the wound myiasis by Wohlfahrtia magnifica in domestic animals

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Use of equine pericardium sheet (lyomesh®) as dura mater substitute in endoscopic endonasal transsphenoidal surgery

Objective: The aim of this study was to describe the use of equine pericardium sheet (Lyomesh®) as dural substitute for sellar reconstruction after endoscopic endonasal transsphenoidal surgery for the removal of pituitary adenomas. Methods: We reviewed data of patients that underwent surgery by means of an endoscopic endonasal transsphenoidal approach for the removal of pituitary adenomas over a 12-months period, starting in May 2012, i.e. when we adopted Lyomesh® (Audio Technologies, Piacenza, Italy) an equine pericardium sheet, as dura mater substitute. Results: During the 12-months period evaluated, we performed an endoscopic endonasal transsphenoidal operation for a variety of pituitary lesions on 102 consecutive patients. Among these, in 12 patients (9.4%) harboring a pituitary adenoma, the implant of the pericardium sheet was used. Four patients (33.3%) presented a small intraoperative cerebrospinal fluid (CSF) leak; in these cases the Lyomesh® was placed intradurally with fibrin glue and, thereafter, several layers were positioned in extradural space. In 8 other subjects without any evidence of CSF leak, the dural substitute was placed intradurally and fibrin glue was injected intradurally to hold the material in place. Conclusions: Even if based on a relatively small patient series, our experience demonstrated that the use of equine pericardium sheet (Lyomesh®) as dura mater substitute in transsphenoidal surgery is safe and biocompatible, as compared with other dural substitutes

Persistent photoconductivity in 2-dimensional electron gases at different oxide interfaces

We report on the transport characterization in dark and under light irradiation of three different interfaces: LaAlO3/SrTiO3, LaGaO3/SrTiO3, and the novel NdGaO3/SrTiO3 heterostructure. All of them share a perovskite structure, an insulating nature of the single building blocks, a polar/non- polar character and a critical thickness of four unit cells for the onset of conductivity. The interface structure and charge confinement in NdGaO3/SrTiO3 are probed by atomic-scale- resolved electron energy loss spectroscopy showing that, similarly to LaAlO3/SrTiO3, extra electronic charge confined in a sheet of about 1.5 nm in thickness is present at the NdGaO3/SrTiO3 interface. Electric transport measurements performed in dark and under radiation show remarkable similarities and provide evidence that the persistent perturbation induced by light is an intrinsic peculiar property of the three investigated oxide-based polar/non-polar interfaces. Our work sets a framework for understanding the previous contrasting results found in literature about photoconductivity in LaAlO3/SrTiO3 and highlights the connection between the origin of persistent photoconductivity and the origin of conductivity itself. An improved understanding of the photo- induced metastable electron-hole pairs might allow to shed a direct light on the complex physics of this system and on the recently proposed perspectives of oxide interfaces for solar energy conversion.Comment: 11 pages, 7 figure