Search results for "fibroblast"
showing 10 items of 667 documents
Transcriptome Analysis of Ullrich Congenital Muscular Dystrophy Fibroblasts Reveals a Disease Extracellular Matrix Signature and Key Molecular Regula…
2015
Background Collagen VI related myopathies encompass a range of phenotypes with involvement of skeletal muscle, skin and other connective tissues. They represent a severe and relatively common form of congenital disease for which there is no treatment. Collagen VI in skeletal muscle and skin is produced by fibroblasts. Aims & Methods In order to gain insight into the consequences of collagen VI mutations and identify key disease pathways we performed global gene expression analysis of dermal fibroblasts from patients with Ullrich Congenital Muscular Dystrophy with and without vitamin C treatment. The expression data were integrated using a range of systems biology tools. Results were validat…
Clinical heterogeneity in infantile galactosialidosis
1987
A new case of infantile galactosialidosis is presented. The condition was diagnosed when the patient was 4 months of age and she died at 20 months. She exhibited some of the symptoms of classical infantile galactosialidosis but no corneal clouding, cherry-red macular spot or limitation of joint mobility. Sonographic examination showed large kidneys and thickened cardiac septa, two symptoms as yet undescribed in this disorder. Urinary oligosaccharide analysis gave grossly pathological results and subsequent fibroblast enzyme analysis showed a deficiency of alpha-neuraminidase and beta-galactosidase. The patient's clinical features are compared with the few cases so far described in the liter…
Desmoplastic fibroblastoma (collagenous fibroma) of the oral cavity
2016
Desmoplastic fibroblastoma is benign soft tissue tumor, with fibroblastic or myofibroblastic origin, that rarely occurs in oral cavity. We reported the case of a 56-year-old man who presented a tumor in the left mandibular alveolar ridge, with slow and asymptomatic growth, with no osseous involvement. The tumor was sessile with lobulated surface, covered by healthy mucosa with erythematous areas. The lesion was excised and specimens sent to histopathology and immunohistochemistry. Histopathological exam showed a non-encapsulated fibroblastic proliferation, characterized by myofibroblasts, spindle and stellate fibroblasts with large or oval nuclei and bi or tri nucleation, immersed in an abu…
Fibroblast and Myofibroblast Participation in Malignant Fibrous Histiocytoma (MFH) of Bone
1989
Eight malignant fibrous histiocytomas (MFH) of bone were studied with immunohistochemistry and electron microscopy. Ultrastructurally, fibroblasts and myofibroblasts were the main tumor cells in four cases and abundant in two other cases; these cells showed immunohistochemical positivity to alpha 1-antitrypsin, vimentin and anti-muscle antigen (HHF 35). Moreover, histiocytic-like tumor cells were electron-microscopically detected in four cases, being the main tumor cell type in two of the cases; immunohistochemically these cells expressed positivity to alpha-1-antichymotrypsin (A1ACT), alpha-1-antitrypsin (A1AT) and vimentin. Present results confirm the cellular heterogeneity of MFH of bone…
Fibronectin as an adjuvant in the diagnosis of oral inflammatory myofibroblastic tumor
2009
Inflammatory myofibroblastic tumor is a distinctive lesion composed of myofibroblastic spindle shaped cells accompanied by inflammatory infiltrate that may arise in various organs. It is believed to be a noneoplastic inflammatory condition, although this is still controversial. The recognition of inflammatory myofibroblastic tumor as an entity is important especially to avoid unnecessary surgery. A few cases have been reported in the oral cavity. This report primarily presents a case of inflammatory myofibroblastic tumor that arose in the floor of mouth of a 23-year-old woman. The proliferating spindle cells were immunoreactive for vimentin, smooth muscle actin, and muscle specific actin an…
Expression of osteopontin messenger RNA and protein in rheumatoid arthritis: Effects of osteopontin on the release of collagenase 1 from articular ch…
2000
Objective Osteopontin (OPN) is an extracellular matrix protein that has been implicated in the interactions between tumor cells and host matrix, including those involved in invasion and spread of tumor cells. Because joint destruction in rheumatoid arthritis (RA) is mediated by the invasive growth of synovial tissue through its attachment to cartilage, we examined the expression of OPN in the synovia of patients with RA and the effect of OPN on the production of collagenase 1 in rheumatoid synovial fibroblasts and articular chondrocytes. Methods The expression of OPN messenger RNA (mRNA) and protein in synovia from 10 RA patients was examined by in situ hybridization and immunohistochemistr…
Role of MUC1 in idiopathic pulmonary fibrosis
2016
Background: Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive and irreversible form of fibrotic intersticial lung disease, characterized by uncontrolled fibroblast proliferative processes and alveolar type II epithelial dysfunction. MUC1 is considered as oncogenic molecule by altering signaling pathways involved in cellular proliferation and cell death. Objective: To analyze the implication of MUC1 in IPF Methods: Lung tissue from 14 healthy and 14 IPF patients was obtained. The expression of MUC1 cytoplasmic tail (CT) and its phosphorylation forms in T-1224 and Y-1229 residues were analyzed by western blot and immunohistochemistry. The effect of MUC1 on TGFβ1-Iinduced epithelia…
Low level 809-nm diode laser-induced in vitro stimulation of the proliferation of human gingival fibroblasts
2002
Background and Objective The authors investigated the effects of low level laser irradiation on the proliferation rate of human gingival fibroblasts (HGF) in vitro. Study Design/Materials and Methods HGF were obtained from gingival connective tissue explants and cultured under standard conditions. 110 cell cultures in their logarithmic growth phase were spread on 96-well tissue culture plates and were irradiated at energy fluences of 1.96–7.84 J/cm2. Another 110 cultures served as control. An 809-nm semiconductor laser operated at a power output of 10 mW in the cw-mode was used. The time of exposure varied between 75 and 300 seconds. Laser treatment was performed alternatively once, twice, …
Role of IL-11 in vascular function of pulmonary fibrosis patients
2019
Pulmonary hypertension (PH) in idiopathic pulmonary fibrosis (IPF) portends a poor prognosis. Currently, no therapy can improve survival of patients diagnosed with this disease. IL-11 molecular pathway is over-expressed in proliferative disorders, however, its role in PH- associated IPF is unknown. The aim of this study was to evaluated the expression of IL-11 in IPF patients with or without PH. Also we hypothesized that the stimulation of pulmonary artery smooth muscle cells (PASMCs) and human pulmonary artery microvascular endothelial cells (HMVEC-L) with IL-11 induced the transformation into invasive myofibroblast. Human pulmonary artery rings, parenchyma tissue, broncho-alveolar lavage …