@article {10591, title = {Complement Protein C1q Binds to Hyaluronic Acid in the Malignant Pleural Mesothelioma Microenvironment and Promotes Tumor Growth.}, journal = {Front Immunol}, volume = {8}, year = {2017}, month = {2017}, pages = {1559}, abstract = {

C1q is the first recognition subcomponent of the complement classical pathway, which acts toward the clearance of pathogens and apoptotic cells. C1q is also known to modulate a range of functions of immune and non-immune cells, and has been shown to be involved in placental development and sensorial synaptic pruning. We have recently shown that C1q can promote tumor by encouraging their adhesion, migration, and proliferation in addition to angiogenesis and metastasis. In this study, we have examined the role of human C1q in the microenvironment of malignant pleural mesothelioma (MPM), a rare form of cancer commonly associated with exposure to asbestos. We found that C1q was highly expressed in all MPM histotypes, particularly in epithelioid rather than in sarcomatoid histotype. C1q avidly bound high and low molecular weight hyaluronic acid (HA) its globular domain. C1q bound to HA was able to induce adhesion and proliferation of mesothelioma cells (MES) enhancement of ERK1/2, SAPK/JNK, and p38 phosphorylation; however, it did not activate the complement cascade. Consistent with the modular organization of the globular domain, we demonstrated that C1q may bind to HA through ghA module, whereas it may interact with human MES through the ghC. In conclusion, C1q highly expressed in MPM binds to HA and enhances the tumor growth promoting cell adhesion and proliferation. These data can help develop novel diagnostic markers and molecular targets for MPM.

}, issn = {1664-3224}, doi = {10.3389/fimmu.2017.01559}, author = {Agostinis, Chiara and Vidergar, Romana and Belmonte, Beatrice and Mangogna, Alessandro and Amadio, Leonardo and Geri, Pietro and Borelli, Violetta and Zanconati, Fabrizio and Tedesco, Francesco and Confalonieri, Marco and Tripodo, Claudio and Kishore, Uday and Bulla, Roberta} } @article {1641, title = {Corticosteroids do not cause harmful increase of viral load in severe H1N1 virus infection.}, journal = {Intensive Care Med}, volume = {36}, year = {2010}, month = {2010 Oct}, pages = {1780-1}, keywords = {Adult, Antiviral Agents, Bronchoalveolar Lavage Fluid, Extracorporeal Membrane Oxygenation, Glucocorticoids, Humans, Influenza A Virus, H1N1 Subtype, Influenza, Human, Male, Methylprednisolone, Oseltamivir, Respiration, Artificial, Severity of Illness Index, Viral Load, World Health Organization}, issn = {1432-1238}, doi = {10.1007/s00134-010-1964-8}, author = {Confalonieri, Marco and D{\textquoteright}Agaro, Pierlanfranco and Campello, Cesare} } @article {1597, title = {Long-term observational, non-randomized study of enzyme replacement therapy in late-onset glycogenosis type II.}, journal = {J Inherit Metab Dis}, volume = {33}, year = {2010}, month = {2010 Dec}, pages = {727-35}, abstract = {

OBJECTIVES: Type II glycogenosis (GSDII) is a lysosomal storage disorder due to acid alpha-glucosidase (GAA) deficiency. Enzyme replacement therapy (ERT) with human recombinant alpha-glucosidase (rhGAA) has been demonstrated to be effective in the treatment of infantile forms of GSDII, but little information is available concerning late-onset phenotypes. Long-term follow-up studies are not available at present. The aim of this study was to evaluate the ERT long-term effects in late-onset GSDII.

METHODS: Twenty-four patients, including 7 juveniles and 17 adults, received bi-weekly infusion of rhGAA (20 mg/kg) for at least 36 months. Clinical conditions, muscular function (6-min walking test, 6MWT; Walton scale, WS), respiratory function (vital capacity, VC; forced expiratory volume, FEV1; arterial pCO(2)), and muscle enzymes were assessed every 6 months.

RESULTS: The 6MWT improved in both juvenile and adult patients (p = 0.01, p = 0.0002, respectively), as well as in patients with moderate to severe muscle function impairment (WS >3.5; p = 0.002). An overall improvement in WS was also observed (p = 0.0003). VC and FEV1 remained unchanged, while pCO(2) decreased (p = 0.017). Muscle enzymes decreased significantly (p < 0.0001). Two patients (8\%) showed transient secondary events during ERT.

CONCLUSIONS: Long-term ERT with rhGAA was shown to be safe, well tolerated, and effective in improving motor function and in stabilizing respiratory function in late-onset GSDII. The response pattern showed a progressive clinical improvement during the follow-up period in juvenile patients, while in adults it reached and maintained a plateau after the first year of treatment.

}, keywords = {Adolescent, Adult, Age of Onset, Aged, alpha-Glucosidases, Child, Enzyme Replacement Therapy, Female, Follow-Up Studies, Glycogen Storage Disease Type II, Humans, Male, Middle Aged, Observation, Time Factors, Treatment Outcome, Young Adult}, issn = {1573-2665}, doi = {10.1007/s10545-010-9201-8}, author = {Bembi, Bruno and Pisa, Federica Edith and Confalonieri, Marco and Ciana, Giovanni and Fiumara, Agata and Parini, Rossella and Rigoldi, Miriam and Moglia, Arrigo and Costa, Alfredo and Carlucci, Annalisa and Danesino, Cesare and Pittis, Maria Gabriela and Dardis, Andrea and Ravaglia, Sabrina} }