Search results for "Myocyte"
showing 10 items of 248 documents
Perlecan is critical for heart stability
2008
Aims Perlecan is a heparansulfate proteoglycan found in basement membranes, cartilage, and several mesenchymal tissues that form during development, tumour growth, and tissue repair. Loss-of-function mutations in the perlecan gene in mice are associated with embryonic lethality caused primarily by cardiac abnormalities probably due to hemopericards. The aim of the present study was to investigate the mechanism underlying the early embryonic lethality and the pathophysiological relevance of perlecan for heart function. Methods and results Perlecan-deficient murine embryonic stem cells were used to investigate the myofibrillar network and the electrophysiological properties of single cardiomy…
Isolation and Characterization of CD276+/HLA-E+ Human Subendocardial Mesenchymal Stem Cells from Chronic Heart Failure Patients: Analysis of Differen…
2012
Mesenchymal stem cells (MSCs) are virtually present in all postnatal organs as well as in perinatal tissues. MSCs can be differentiated toward several mature cytotypes and interestingly hold potentially relevant immunomodulatory features. Myocardial infarction results in severe tissue damage, cardiomyocyte loss, and eventually heart failure. Cellular cardiomyoplasty represents a promising approach for myocardial repair. Clinical trials using MSCs are underway for a number of heart diseases, even if their outcomes are hampered by low long-term improvements and the possible presence of complications related to cellular therapy administration. Therefore, elucidating the presence and role of MS…
ACUTE INFANTILE SPINAL MUSCULAR ATROPHY
1990
Biopsy as well as autopsy studies of a child who died 8 weeks after birth from the acute infantile form of spinal muscular atrophy revealed classical morphological changes, including degeneration and loss of motoneurons in the spinal cord, loss of large myelinated fibres in anterior roots and neurogenic atrophy in muscle. New ultrastructural findings include massive muscle cell elimination by apoptosis with the formation of membrane-bound muscle cell fragments, apoptotic bodies. In addition, numerous immature muscle fibres were observed. The morphological findings raise the possibility that in a severely growth-retarded muscle, the process of muscle cell apoptosis removes the peripheral tar…
Current understanding of somatic stem cells in leiomyoma formation.
2014
Objective To provide a detailed summary of current scientific knowledge of somatic stem cells (SSCs) in murine and human myometrium and their putative implication in leiomyoma formation, as well as to establish new therapeutic options. Design Pubmed and Scholar One manuscripts were used to identify the most relevant studies on SSCs and their implications in human myometrium and leiomyomas. Setting University research laboratory–affiliated infertility clinic. Patient(s) Not applicable. Intervention(s) Not applicable. Main Outcome Measure(s) Not applicable. Result(s) Despite numerous publications on SSCs, it was not until 2007 that scientific evidence based on the use of 5-bromo-2′-deoxyuridi…
Therapeutic targets for overactive bladder other than smooth muscle
2015
For a long time, our concepts of regulation of urinary bladder function in health and disease as well as of the target structures of therapeutics have focused on detrusor smooth muscle cells. However, other structures including urothelium, afferent nerves and bladder blood vessels may also be important in pathophysiology and its treatment.Based on a selective review of literature, we discuss the role of urothelium, afferent nerve fibers and bladder blood vessels in bladder pathophysiology and as targets for treatment.There is solid evidence now that multiple anatomical structures within the urinary bladder contribute to the regulation of its function and hence may be targets for established…
Desmin-related neuromuscular disorders
1995
Desmin, the intermediate filament protein of skeletal muscle fibers, cardiac myocytes, and certain smooth muscle cells, is a member of the cytoskeleton linking Z-bands with the plasmalemma and the nucleus. The pathology of desmin in human neuromuscular disorders is always marked by increased amounts, diffusely or focally. Desmin is highly expressed in immature muscle fibers, both during fetal life and regeneration as well as in certain congenital myopathies, together with vimentin. Desmin is also enriched in neonatal myotonic dystrophy and small fibers in infantile spinal muscular atrophy. Focal accretion of desmin may be twofold, in conjunction with certain inclusion bodies, cytoplasmic an…
A novel in vitro model for the study of plaque development in atherosclerosis
2006
SummaryFor the study of atherogenesis in vitro, coculture systems have been devised, in which two or more cell types can be cultured in close contact to each other. Herein, we describe a novel in vitro model that aims at the simulation of the morphology ofa normal muscular artery allowing for the study of the initial events in atherosclerosis. Usinga modified fibrin gel as a scaffold for the coculture of endothelial cells (ECs) and smooth muscle cells (SMCs), we generated an autologous in vitro model with a multilayer growth of SMCs (intima-like structure) covered by an endothelium. The production of extracellular matrix (ECM) could be visualized histologically and verified by (i) ascorbic-…
Subendothelial infiltration of neutrophil granulocytes and liberation of matrix-destabilizing enzymes in an experimental model of human neo-intima.
2008
SummaryIt was the objective of this study to examine the role of human neutrophil granulocytes (PMN) in an in-vitro model of human neo-intima developed for the study of atherosclerosis. Human granulocytes were subjected to a co-culture model of human endothelial and smooth muscle cells. Subendothelial lipid accumulation was achieved by addition of native LDL to the culture medium. Tissue samples were analyzed by immunohistochemistry and scanning/transmission electron microscopy, and culture supernatants were examined for the presence of interleukin- 8 (IL-8), MCP-1, GRO-α, elastase and matrixmetalloproteinase-8 (MMP-8). Following addition of 2 mg/ml LDL, adherence, transmigration and infilt…
Primary desminopathies.
2007
• Introduction • Desmin is an essential component of the extrasarcomeric cytoskeleton in striated muscle cells • Distal myopathy,cardiac arrhythmias,cardiomyopathy:classical criteria of primary desminopathies • Sub-sarcolemmal and cytoplasmic desmin-positive protein aggregates:the morphological hallmark of primary and secondary desminopathies • The spectrum of pathogenic desmin gene mutations • The molecular pathogenesis of primary desminopathies: some answers gained,but even more questions raised • Diagnostic work-up to distinguish primary from secondary desminopathies • Treatment and clinical management of primary desminopathy patients Abstract Mutations of the human desmin gene o…
0331 : Pathophysiology of the ubiquitine ligase E3, PDZRN3, in the development of dilated cardiomyopathies
2015
Dilated cardiomyopathy is a major cause of heart failure with a poor prognostic. Molecular mechanisms underlying the transition toward the dilated phenotype are still not known. In heart, individual cardiomyocytes connect some with the others via their extremities by junctional platform (Intercalated Discs, ID) crucial for the mechanical coupling and the anisotropic conduction of the electric signal. In this project, we are interested in an Ubiquitine ligase E3 called PDZRN3, which is expressed and regulated in cardiomyocytes during their maturation. We have previously identified PDZRN3 involvement in the the Wnt Planar Cell Polarity (Wnt/PCP) signaling in vascular morphogenesis. In the hea…