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Procedure: Gastroschisis Repair

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Total 132 results found since Jan 2013.

Successful open abdomen treatment for multiple ischemic duodenal perforated ulcers in dermatomyositis
Conclusions: In patients with dermatomyositis, when clinical findings and symptoms suggest abdominal vasculitis, it is very important to be aware of the risk of bowel and particularly duodenal perforations. Open abdomen treatment favors control of contamination by gastrointestinal contents, offers temporary abdominal closure, helps ICU care and delays definitive surgery.
Source: World Journal of Emergency Surgery - August 30, 2014 Category: Emergency Medicine Authors: Roberta VillaStefano CostaSibilla FocchiCarlo CorbelliniMassimo VigorelliEttore Contessini Avesani Source Type: research

Amphiphysin 2 (BIN1) in physiology and diseases.
Abstract Amphiphysin 2, also named bridging integrator-1 (BIN1) or SH3P9, has been recently implicated in rare and common diseases affecting different tissues and physiological functions. BIN1 downregulation is linked to cancer progression and also correlates with ventricular cardiomyopathy and arrhythmia preceding heart failure. Increased BIN1 expression is linked to increased susceptibility for late-onset Alzheimer's disease. In addition, altered splicing may account for the muscle component of myotonic dystrophies, while recessive germinal mutations cause centronuclear myopathy. Despite undoubtedly underlining ...
Source: Molecular Medicine - March 5, 2014 Category: Molecular Biology Authors: Prokic I, Cowling BS, Laporte J Tags: J Mol Med (Berl) Source Type: research

T-tubule dysferlin stabilizes Ca2+ signaling Physiology
Dysferlinopathies, most commonly limb girdle muscular dystrophy 2B and Miyoshi myopathy, are degenerative myopathies caused by mutations in the DYSF gene encoding the protein dysferlin. Studies of dysferlin have focused on its role in the repair of the sarcolemma of skeletal muscle, but dysferlin’s association with calcium (Ca2+) signaling proteins...
Source: Proceedings of the National Academy of Sciences - December 17, 2013 Category: Science Authors: Kerr, J. P., Ziman, A. P., Mueller, A. L., Muriel, J. M., Kleinhans-Welte, E., Gumerson, J. D., Vogel, S. S., Ward, C. W., Roche, J. A., Bloch, R. J. Tags: Biological Sciences Source Type: research

Chronic binge alcohol (CBA) consumption increases cell death and alters myogenic gene expression in skeletal muscle satellite cells
In this study, we have examined the effects of CBA on satellite cell proliferative and differentiation potential. SC were isolated from CBA rhesus macaques and seeded on collagen plates for 14 days. The cells were cultured for the first 7 days in proliferation media (Ham's F12 with 20% fetal bovine serum and 10 ng/ml of epidermal growth factor) and the next 7 days in differentiation media (Ham's F12 with 2% horse serum). SC isolated from skeletal muscle of CBA macaques showed significantly increased apoptosis and necrosis, and no change in autophagy during ex vivo proliferation. In addition, SCs isolated from skeletal mus...
Source: Alcohol - November 1, 2013 Category: Addiction Authors: L. Simon, N. LeCapitaine, P. Berner, T. Dodd, C. Vande Stouwe, P. Molina Tags: Abstracts Source Type: research

Full‐length dysferlin expression driven by engineered human dystrophic blood derived CD133+ stem cells
The protein dysferlin is abundantly expressed in skeletal and cardiac muscles, where its main function is membrane repair. Mutations in the dysferlin gene are involved in two autosomal recessive muscular dystrophies: Miyoshi myopathy and limb‐girdle muscular dystrophy type 2B. Development of effective therapies remains a great challenge. Strategies to repair the dysferlin gene by skipping mutated exons, using antisense oligonucleotides (AONs), may be suitable only for a subset of mutations, while cell and gene therapy can be extended to all mutations. AON‐treated blood‐derived CD133+ stem cells isolated from patients...
Source: FEBS Journal - October 8, 2013 Category: Research Authors: Mirella Meregalli, Claire Navarro, Clementina Sitzia, Andrea Farini, Erica Montani, Nicolas Wein, Paola Razini, Cyriaque Beley, Letizia Cassinelli, Daniele Parolini, Marzia Belicchi, Dario Parazzoli, Luis Garcia, Yvan Torrente Tags: Original Article Source Type: research

P.9.3 Repair of mutant nebulin transcripts by exon exchange
The nebulin gene (NEB) has 183 exons encoding transcripts up to 26kb in length. Mutations found in NEB are dispersed throughout the gene. Mutations cause autosomal recessive nemaline myopathy, distal myopathy and core-rod myopathy, for which no therapy is available. The size of NEB limits the options of gene therapy development. Thus, our research has focused on methods correcting the mutation carrying transcripts. The exon exchange method can be developed to exchange a mutated exon by spliceosome mediated RNA trans-splicing occurring between the target pre-mRNA and a carefully designed therapy molecule, PTM (Pre-Trans-spl...
Source: Neuromuscular Disorders - September 16, 2013 Category: Neurology Authors: J. Laitila, J.J. Dowling, K. Pelin Source Type: research

P.5.1 Sarcolemmal repair and reorganization of microtubule
We reported that affixin, β- and γ-actin accumulated at the sarcolemmal injury site of wild-type mice in response to membrane rapture. α-tubulin and microtubule-associated proteins have been reported as dysferlin binding partners, however, their involvement in sarcolemmal repair remains unclear.The purpose of this study is to examine involvement of microtubule in sarcolemmal repair. To clarify molecular behavior of α-tubulin in sarcolemmal repair, GFP2-tagged human α-tubulin was expressed in mouse FDB muscle (C57BL/6J and dysferlin-deficient SJL) by electroporation. Membrane wound-repair assay of single myofiber was p...
Source: Neuromuscular Disorders - September 16, 2013 Category: Neurology Authors: C. Matsuda, K. Miyake, T. Imamura, N. Araki, I. Nishino, Y.K. Hayashi Source Type: research

Full‐Length Dysferlin Expression Driven by Engineered Human Dystrophic Blood‐Derived CD133+ Stem Cells
This article is protected by copyright. All rights reserved.
Source: FEBS Journal - September 13, 2013 Category: Research Authors: Mirella Meregalli, Claire Navarro, Clementina Sitzia, Andrea Farini, Erica Montani, Nicolas Wein, Paola Razini, Cyriaque Beley, Letizia Cassinelli, Daniele Parolini, Marzia Belicchi, Dario Parazzoli, Luis Garcia, Yvan Torrente Tags: Original Article Source Type: research

P.9.3 Repair of mutant nebulin transcripts by exon exchange
The nebulin gene (NEB) has 183 exons encoding transcripts up to 26kb in length. Mutations found in NEB are dispersed throughout the gene. Mutations cause autosomal recessive nemaline myopathy, distal myopathy and core-rod myopathy, for which no therapy is available. The size of NEB limits the options of gene therapy development. Thus, our research has focused on methods correcting the mutation carrying transcripts. The exon exchange method can be developed to exchange a mutated exon by spliceosome mediated RNA trans-splicing occurring between the target pre-mRNA and a carefully designed therapy molecule, PTM (Pre-Trans-spl...
Source: Neuromuscular Disorders - September 7, 2013 Category: Neurology Authors: J. Laitila, J.J. Dowling, K. Pelin Source Type: research

P.5.1 Sarcolemmal repair and reorganization of microtubule
We reported that affixin, β- and γ-actin accumulated at the sarcolemmal injury site of wild-type mice in response to membrane rapture. α-tubulin and microtubule-associated proteins have been reported as dysferlin binding partners, however, their involvement in sarcolemmal repair remains unclear.The purpose of this study is to examine involvement of microtubule in sarcolemmal repair. To clarify molecular behavior of α-tubulin in sarcolemmal repair, GFP2-tagged human α-tubulin was expressed in mouse FDB muscle (C57BL/6J and dysferlin-deficient SJL) by electroporation. Membrane wound-repair assay of single myofiber was p...
Source: Neuromuscular Disorders - September 7, 2013 Category: Neurology Authors: C. Matsuda, K. Miyake, T. Imamura, N. Araki, I. Nishino, Y.K. Hayashi Source Type: research

Autocrine and immune cell‐derived BDNF in human skeletal muscle: implications for myogenesis and tissue regeneration
In conclusion, BDNF is an autocrine factor for skeletal muscle cells and may regulate human myogenesis. Furthermore, the preferential localization of BDNF‐producing immune cells near p75NTR‐positive regenerating myofibres suggests that immune cell‐derived BDNF may sustain tissue repair in inflamed muscle. Copyright © 2013 Pathological Society of Great Britain and Ireland. Published by John Wiley & Sons, Ltd.
Source: The Journal of Pathology - August 12, 2013 Category: Pathology Authors: Emanuela Colombo, Francesco Bedogni, Isabella Lorenzetti, Nicoletta Landsberger, Stefano C. Previtali, Cinthia Farina Tags: Original Paper Source Type: research

Autocrine and immune cell derived BDNF in human skeletal muscle: implications for myogenesis and tissue regeneration
In conclusion, BDNF is an autocrine factor for skeletal muscle cells and may regulate human myogenesis. Furthermore, the preferential localisation of BDNF producing immune cells near p75NTR positive regenerating myofibers suggests that immune cell derived BDNF may sustain tissue repair in inflamed muscle.
Source: The Journal of Pathology - June 14, 2013 Category: Pathology Authors: Emanuela Colombo, Francesco Bedogni, Isabella Lorenzetti, Nicoletta Landsberger, Stefano C. Previtali, Cinthia Farina Tags: Original Article Source Type: research