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2. Mechanisms underlying Andersen's syndrome pathology in skeletal muscle are revealed in human myotubes

Author

Sacconi, S., Simkin, D., Arrighi, N., Chapon, F., Larroque, M.M., Vicart, S., Sternberg, D., Fontaine, B., Barhanin, J., Desnuelle, C., and Bendahhou, S.

Subjects
Glycogenosis -- Development and progression, Glycogenosis -- Genetic aspects, Membrane potentials -- Health aspects, Muscles -- Health aspects, Biological sciences
Abstract

Andersen's syndrome is a rare disorder that has been defined with a triad: periodic paralysis, cardiac arrhythmia, and development anomalies. Muscle weakness has been reported in two-thirds of the patients. KCNJ2 remains the only gene linked to Andersen's syndrome; this gene encodes for the [alpha]-subunit of the strong inward-rectifier [K.sup.+] channel Kir2.1. Several studies have shown that Andersen's syndrome mutations lead to a loss of function of the [K.sup.+] channel activity in vitro. However, ex vivo studies on isolated patient muscle tissue have not been reported. We have performed muscle biopsies of controls and patients presenting with clinically and genetically defined Andersen's syndrome disorder. Myoblasts were cultured and characterized morphologically and functionally using the whole cell patch-clamp technique. No morphological difference was observed between Andersen's syndrome and control myoblasts at each passage of the cell culture. Cellular proliferation and viability were quantified in parallel with direct cell counts and showed no difference between control and Andersen's syndrome patients. Moreover, our data show no significant difference in myoblast fusion index among Andersen's syndrome and control patients. Current recordings carried out on myotubes revealed the absence of an inwardly rectifying [Ba.sup.2+]-sensitive current in affected patient cells. One consequence of the Ik1 current loss in Andersen's syndrome myotubes is a shift of the resting membrane potential toward depolarizing potentials. Our data describe for the first time the functional consequences of Andersen's syndrome mutations ex vivo and provide clues to the [K.sup.+] channel pathophysiology in skeletal muscle. skeletal muscle; Andersen's syndrome; potassium ion channels doi: 10.1152/ajpcell.00519.2008

Published
2009

10. Radical Cystectomy for Transitional Cell Carcinoma with Orthotopic Neobladder in Renal Transplant Recipients: Surgical Procedure, Functional and Therapeutic Evaluation

Author

Zani, Danilo, Simeone, Claudio, Arrighi, N., Perucchini, L., Antonelli, A., Setti, C., Sandrini, S., Cancarini, G., and Cunico, S. C.

Subjects
General Medicine
Abstract

Introduction Transitional Cell Carcinoma (TCC) shows a high prevalence in renal tranplant recipients, nevertheless urinary reconstruction by way of orthotopic neobladder is rarely reported. Patients and Methods Two kidney transplant patients (1 man and 1 woman) affected by TCC underwent radical cystectomy and urinary reconstruction by neobladder according to the “Vescica Ileale Padovana” technique. Results and Discussion No patients claimed incontinence or urine retention. The spherical rebuilding assured an adequate bladder capacity and a low endovesical pressure. After 48 months one patient died because of disease progression. The first relapse was detected in ureter two years after cystectomy. The other patient is still alive after 40 months and in good health conditions. Conclusions This anecdotal report suggests that the “Vescica Ileale Padovana” technique could be useful in renal transplant recipients with TCC. A possible role of Rapamycin on our results cannot yet be excluded.

Published
2009

11. Renal cell carcinoma associated with incidental presentation of renal artery aneurysm: surgical management

Author

Zani, Danilo, Arrighi, N., Antonelli, A., Cunico, S. C., and Simeone, Claudio

Subjects
03 medical and health sciences, 0302 clinical medicine, 030220 oncology & carcinogenesis, 030232 urology & nephrology, General Medicine, urologic and male genital diseases
Abstract

The Renal Artery Aneurysm (RAA) is a relatively uncommon vascular lesion. A renal artery disease coexisting in patients with Renal Cell Carcinoma (RCC) is an even more infrequent clinical presentation. We reported on the treatment of a rare case of incidentally intraoperative renal artery aneurysm discovered during a nephron-sparing surgery for RCC. After the surgery the patient did not need hypertension therapy any longer. This event is well-known, in fact a number of possible contributions to a renin-mediated hypertension management has been postulated.

Published
2008

12. Characterization of adipocytes derived from fibro/adipogenic progenitors resident in human skeletal muscle

Author

Arrighi, N, Moratal, C, Clément, N, Giorgetti-Peraldi, S, Peraldi, P, Loubat, A, Kurzenne, J-Y, Dani, C, CHOPARD, ANGELE, Dechesne, C A, Institut de Biologie Valrose (IBV), Université Nice Sophia Antipolis (... - 2019) (UNS), COMUE Université Côte d'Azur (2015-2019) (COMUE UCA)-COMUE Université Côte d'Azur (2015-2019) (COMUE UCA)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS)-Université Côte d'Azur (UCA), Hôpitaux Pédiatriques de Nice Lenval (CHU-Lenval), Centre Hospitalier Universitaire de Nice (CHU Nice), Dynamique Musculaire et Métabolisme (DMEM), Institut National de la Recherche Agronomique (INRA)-Université de Montpellier (UM), Université Nice Sophia Antipolis (1965 - 2019) (UNS), ProdInra, Archive Ouverte, COMUE Université Côte d'Azur (2015-2019) (COMUE UCA)-COMUE Université Côte d'Azur (2015-2019) (COMUE UCA)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Université Côte d'Azur (UCA)-Centre National de la Recherche Scientifique (CNRS), Université Côte d'Azur (UCA)-Université Côte d'Azur (UCA)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), and Université de Montpellier (UM)-Institut National de la Recherche Agronomique (INRA)

Subjects
insulin, Adipogenesis, fibre musculaire, Stem Cells, [SDV]Life Sciences [q-bio], skeletal myocytes, Cell Differentiation, muscle squelettique, Fibroblasts, adipocyte, [SDV] Life Sciences [q-bio], voluntary muscle, Adipocytes, Humans, Original Article, lipocytes, Muscle, Skeletal, Cells, Cultured, insuline
Abstract

A population of fibro/adipogenic but non-myogenic progenitors located between skeletal muscle fibers was recently discovered. The aim of this study was to determine the extent to which these progenitors differentiate into fully functional adipocytes. The characterization of muscle progenitor-derived adipocytes is a central issue in understanding muscle homeostasis. They are considered as being the cellular origin of intermuscular adipose tissue that develops in several pathophysiological situations. Here fibro/adipogenic progenitors were isolated from a panel of 15 human muscle biopsies on the basis of the specific cell-surface immunophenotype CD15(+)/PDGFRα(+)CD56(-). This allowed investigations of their differentiation into adipocytes and the cellular functions of terminally differentiated adipocytes. Adipogenic differentiation was found to be regulated by the same effectors as those regulating differentiation of progenitors derived from white subcutaneous adipose tissue. Similarly, basic adipocyte functions, such as triglyceride synthesis and lipolysis occurred at levels similar to those observed with subcutaneous adipose tissue progenitor-derived adipocytes. However, muscle progenitor-derived adipocytes were found to be insensitive to insulin-induced glucose uptake, in association with the impairment of phosphorylation of key insulin-signaling effectors. Our findings indicate that muscle adipogenic progenitors give rise to bona fide white adipocytes that have the unexpected feature of being insulin-resistant.

Published
2015

13. EFFICACY OF FLOSEAL® VERSUS TACHOSI L® VERSUS NO HEMOSTATIC AGENTS FOR NEPHRON SPARING SURGERY : A PROSPECTIVE multicenter STUDY (RECORd PROJECT)

Author

Antonelli A, Carini M, Arrighi N, Bianchi G, Cosciani Cunico S, Ficarra V, Fiori C, Masieri L, Medica M, Mirone V, Pinzi N, Porpiglia F, Rocco F, Rovereto B, Serni, Simeone C, Terrone C, Volpe A, Zattoni F, Minervini A., MARTORANA, GIUSEPPE, SCHIAVINA, RICCARDO, Antonelli A, Carini M, Arrighi N, Bianchi G, Cosciani Cunico S, Ficarra V, Fiori C, Martorana G, Masieri L, Medica M, Mirone V, Pinzi N, Porpiglia F, Rocco F, Rovereto B, SCHIAVINA R., Serni, Simeone C, Terrone C, Volpe A, Zattoni F, and Minervini A

Subjects
EFFICACY OF FLOSEAL® VERSUS TACHOSI L® VERSUS NO HEMOSTATIC AGENTS
Published
2012

14. Predictive factors of positive surgical margins in nephron sparing surgery: A prospective multicenter comparative study (RECORd1 project)

Author

Minervini, Andrea, Antonelli, A., Bertolo, R., Bianchi, G., Fusco, F., Siena, Giampaolo, Fiori, C., Tuccio, Agostino, Mari, A., Martorana, G., Mirone, V., Morgia, G., Novara, G., Porpiglia, F., Schiavina, R., Arrighi, N., Vittori, Gianni, Terrone, C., Ficarra, V., Carini, Marco, and RECORd project Italy

Subjects
Predictive factors , Positive surgical margins , Nephron sparing surgery
Published
2014

15. Simple tumor enucleation and standard partial nephrectomy have similar perioperative results and trifecta outcomes: Comparison based on a matched-pair analysis of 400 patients (RECORd1 project)

Author

Minervini, Andrea, Antonelli, A., Bertolo, R., Bianchi, G., Fusco, F., Siena, Giampaolo, Fiori, C., Giancane, Saverio, Longo, Nicola, Mari, A., Martorana, G., Mirone, V., Rovereto, B., Morgia, G., Novara, G., Porpiglia, F., Schiavina, R., Arrighi, N., Terrone, C., Ficarra, V., Carini, Marco, and RECORd project Italy

Subjects
Simple tumor enucleation ,standard partial nephrectomy, perioperative results, RECORd1 project
Published
2014

16. Efficacy of floseal® vs tachosil® vs no hemostatic agents for partial nephrectomy: A prospective multicenter comparative study (RECORd Project)

Author

Antonelli, A., Minervini, A., Arrighi, N., Bianchi, G., Cosciani Cunico, S., Ficarra, V., Fiori, C., Martorana, G., Lapini, A., Medica, M., Mirone, V., Pinzi, N., Porpiglia, F., Rocco, F., Rovereto, B., Schiavina, R., Serni, S., Simeone, C., Terrone, C., Volpe, A., Zattoni, F., Carini, M., and Record

Subjects
floseal® vs tachosil® , partial nephrectomy: A prospective multicenter comparative study (RECORd
Published
2013

19. EFFICACY OF FLOSEAL* VERSUS TACHOSIL* VERSUS NO HEMOSTATIC AGENTS FOR NEPHRON SPARING SURGERY: A PROSPECTIVE MULTICENTER COMPARATIVE STUDY ( RECORD PROJECT )

Author

Antonelli, A., Carini, M., Arrighi, N., Bianchi, G., Cosciani Cunico, S., Ficarra, V., Fiori, C., Martorana, G., Masieri, L., Medica, M., Mirone, V., Pinzi, N., Porpiglia, F., Rocco, F., Rovereto, B., Schiavina, R., Serni, S., Simeone, C., Terrone, C., Volpe, A., Zattoni, F., and Minervini, A.

Subjects
FLOSEAL, TACHOSI, HEMOSTATIC AGENTS, NEPHRON SPARING SURGERY: (RECORD PROJECT)
Published
2012

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