Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UNIPROT:Q16637 (SMA)
 8,107 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We report 2 cases of Follicular Dendritic Cell Sarcomas of Oral Cavity in two elderly patients. The patients presented with oral cavity tumors. Initial tru-cut biopsies in both cases revealed spindle cell neoplasms. One of them was of low grade malignancy and showed positivity for of Vimentin. Few cells showed positivity for keratin and were negative for S-100 protein, CD 34, CD 68, EMA, SMA, HMB-45. A possible diagnosis of low grade fibrohistiocytic tumor was made. He underwent Lt total maxillectomy. Three years later he presented back with regional nodal metastasis. The regional lymph nodes showed features of follicular dendritic cell sarcoma. The second case revealed high grade spindle cell neoplasm and showed positivity for vimentin and S100 protein and was negative for EMA, keratin, CD-34, desmin, muscle actin and HMB-45. He was offered initial radio-therapy followed by hemifacial resection. The histology along with immuno histochemistry favoured a diagnosis of follicular dendritic cell sarcoma. He presented with local recurrence two months later. Both the cases are discussed in detail.
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PMID:Follicular dendritic cell sarcoma of oral cavity: report of 2 cases. 1788 29

Leiomyosarcomas (LMS) are the most common type of uterine sarcoma. Most LMS have typical histologic features, and variants such as epithelioid LMS, myxoid LMS, LMS with osteoclast-like giant cells and LMS with rhabdoid features occur only rarely. Rhabdoid cells were first described in rhabdoid tumor, a distinctive renal neoplasm of infancy. Such tumors are composed of diffuse proliferation of rhabdoid cells that are round or polygonal in shape with eccentric nuclei, prominent nucleoli and glassy eosinophilic cytoplasm containing hyaline-like inclusion bodies. In the literature, extrarenal localizations of malign rhabdoid tumors have been described in a variety of primary sites such as the central nervous system, liver, skin and soft tissues. These characteristic rhabdoid cells have been reported in sarcomas and carcinomas of various types and in a few cases of uterine sarcomas. The presence of rhabdoid cells in tumors is considered to be a predictor of aggressive tumor behavior. Our case is that of a 56-year-old woman who was admitted to the state hospital with left inguinal mass. Microscopically the tumor was admixed of three different types of cell with spindle, epithelioid or rhabdoid features. Immunopositive cytoplasmic staining for myoglobulin and desmin was seen in rhabdoid cells, and cytokeratin immunopositivity was observed in epithelioid and some rhabdoid cells. Epithelioid cells and spindle cells were also SMA positive. The histopathologic and immunohistochemical findings support the diagnosis of epithelioid LMS with rhabdoid features. We report this very uncommon LMS variant; to the best of our knowledge there are only a few cases in the English literature.
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PMID:Epithelioid leiomyosarcoma with rhabdoid features. 1794 9

Sarcomas are neoplasms of mesenchymal origin, with a predominant cell population mimicking the organization of various soft tissues and/or bones. Previous categorizations also included the possibility of the presence of tissue macrophage-like (histiocytes) neoplasm cells, in a tumour described as malignant fibrous histiocytoma, but this group has been considered as a variety of undifferentiated pleomorphic sarcomas. Although this kind of malignancy is not rare in humans, only few cases have been reported in laboratory animals. We report an unusual single case of spontaneous tumour growth, detected by casual observation, in the left thoracic area of an 18-month-old male laboratory Wistar rat. Both this individual and his ancestors were not exposed to any known carcinogenic substance or radiation, thus suggesting the development of the neoplasm as a spontaneous event. The mass was extracted surgically under general anaesthesia, and slices were examined histologically and immunohistochemically, using photon microscopy. The pathologist reported the presence of a combination of fibroblasts and undifferentiated mesenchymal cells arranged in a storiform pattern. Immunohistochemistry was performed on the tissue using specific antibodies for several proliferation (Ki-67) and differentiation (S-100, CD-34, CD-68, pan-keratin, desmin and smooth muscle actin-SMA) markers. Positive reaction was observed for S-100, Ki-67, CD-68, desmin and SMA (limited) but not for CD-34 or cytokeratin.
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PMID:Casual discovery of a thoracic tumour showing histological features of undifferentiated pleomorphic sarcoma in a male Wistar laboratory rat. 1802 53

As a practical concept of regenerative medicine, we have focused on in vivo tissue engineering utilizing the foreign body reaction. Plastic substrates for valvular leaflet organization, consisting of two pieces assembled with a small aperture were inserted into a microporous polyurethane conduit scaffold. The assembly was placed in the subcutaneous spaces of Japanese white rabbits for 1 month. After the substrates were pulled out from the harvested implant, valve leaflet-shaped membranous tissue was formed inside the tubular scaffold as designed. The valve leaflet was composed of the same collagen-rich tissue, with the absence of any elastic fiber, as that which had ingrown or covered the scaffold. No abnormal collection or infiltration of inflammatory cells in the leaflet and the scaffold could be demonstrated. According to the immunohistochemical staining, the leaflet was comprised of numerous vimentin- or alpha-SMA-positive cells, corresponding to fibroblasts or myofibroblats, but contained no desmin-positive cells. The analysis of the video data of the valve movement showed that, in synchronization with the backward flow in the diastolic phase, the valve closed rapidly and tightly and, in the transition phase of the flow direction, the valve opened smoothly without flapping or hitting the scaffold wall. Using mold designs, consisting of two different plastic substrates and the tubular scaffold, in conjunction with "in body tissue architecture," the complex 3-dimensional autologous conduit-typed Biovalve was developed for the first time.
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PMID:Architecture of an in vivo-tissue engineered autologous conduit "Biovalve". 1807 96

Cells were isolated from four human amniotic membranes, and their biological characteristics analyzed during ex vivo expansion. Morphologically homogenous populations of fibroblast-like cells were obtained from the second or third passage. Under the appropriate culture conditions, these human amniotic membrane-derived mesenchymal cells (HAM) were shown to differentiate into adipocytes, osteocytes, chondrocytes and neuronal cells, as visualized by Oil Red O, von Kossa, alcian blue, anti-Neu N, and anti-Gal C antibody staining, respectively. Immunophenotype analysis of HAM cells revealed the presence of antigens for SSEA-3, SSEA-4, collagen type-I, -II, -III, -IV, -XII, fibronectin, alpha-SMA, vimentin, desmin, cytokeratin18 (CK18), HCAM-1, fibroblast surface protein, and human leukocyte antigen (HLA) ABC. ICAM-1 protein was weakly detectable, and proteins of TRA-1-60, VCAM-1, von Willebrand factor, PECAM-1, and HLA DR were not detected. HAM cells reached senescence after 14.5+/-0.9 passages, over a period of 146.8+/-8.9 days, and underwent an average of 36.9 4.7 population doublings. RT-PCR analysis showed that all four HAM cell lines consistently expressed genes of Oct-4, Rex-1, SCF, NCAM, nestin, BMP-4, GATA-4, HNF-4alpha, vimentin, and CK18, regardless of the passage number. The genes of Brachyury, FGF-5, Pax-6, and BMP2 were never expressed. Strikingly, alpha-fetoprotein (alphaFP), HLA ABC, and HLA DR genes were expressed in an earlier passage but not expressed in later passages. Telomerase activity of two HAM lines was discernable upon the third passage. These observations strongly suggest that HAM might be immune-privileged and, thus, advantageous as therapeutic cells.
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PMID:Ex vivo characteristics of human amniotic membrane-derived stem cells. 1815 18

Bone marrow-derived cells are recruited into tumor vasculature in response to angiogenic signals, and some of the cells within the newly forming tumor vessels are hematopoietic stem cells (HSCs) in origin. Previous studies suggest that bone marrow-derived pericytes are associated with newly formed vessels in tumors. In this study, we used an orthotopic rat glioma model (RT-2/RAG) to examine the contribution of long-term hematopoietic stem cell (LT-HSC)-derived pericytic cells to brain tumor angiogenesis. Mice (RAG-2/KO5.2) were lethally irradiated, and their hematopoietic cells were repopulated by transplantation of double fluorescence-activated cell-sorted LT-HSCs that express green fluorescent protein (GFP+). RT-2/RAG cells were then injected into the striatum of the chimeric mice 6 weeks post-transplantation. The animals were sacrificed 9 days after tumor implantation, and the incorporation and lineage-specific marker expression profile of the GFP+ cells within the growing tumor and tumor periphery were analyzed. LT-HSC-derived GFP+ cells were noted to incorporate onto the surface of tumor vessels within the perivascular space. LT-HSC-derived GFP+ cells express the pericyte progenitor marker, platelet-derived growth factor receptor-beta (PDGFR beta), as well as mature perictyte markers such as nerve/glial antigen 2 proteoglycan (NG2), alpha-smooth muscle actin (alpha SMA), and desmin. These LT-HSC-derived cells may represent a population of progenitor or committed pericytes within the neovascular tree and may play a role in shaping the angio-architecture in the vascular niche of brain tumors.
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PMID:Hematopoietic stem cell-derived pericytic cells in brain tumor angio-architecture. 1824 Sep 55

According to the World Health Organization classification, neoplasms with perivascular epithelioid cell differentiation (PEComas) are mesenchymal tumors composed of histologically and immunohistochemically distinctive PEC. Generally, nearly all PEComas have immunoreactivity for both melanocytic (HMB-45 and/or melan A) and smooth muscle (actin (SMA) and/or desmin) markers. Recently the authors reported that benign clear cell sugar tumor of the lung, one of the PEComas, expressed CD1a. Therefore the purpose of the present study was to investigate the relationship between PEComas and CD1a expression. Nineteen PEComas were obtained, which included angiomyolipoma of the kidney or liver, lymphangiomyomatosis of the uterus or lung and clear cell sugar tumor of the lung. Eighteen tumors had alpha-SMA and HMB-45 expression and 16 had melan A expression. In contrast, all 19 tumors had CD1a expression. The present study confirms CD1a expression in many cases of PEComa. These data suggest that CD1a expression can be an additional new marker for PEComas and also supports the distinct and integrated disease entity of PEComas.
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PMID:CD1a expression in PEComas. 1825 80

The formation of myofibroblasts in valve interstitial cell (VIC) populations contributes to fibrotic valvular disease. We examined myofibroblast differentiation in VICs from porcine aortic valves. In normal valves, cells immunostained for alpha-smooth muscle actin (alpha-SMA, a myofibroblast marker) were rare (0.69 +/- 0.48%), but in sclerotic valves of animals fed an atherogenic diet, myofibroblasts were spatially clustered and abundant (31.2 +/- 6.3%). In cultured VIC populations from normal valves, SMA-positive myofibroblasts were also spatially clustered, abundant (21% positive cells after 1 passage), and stained for collagen type I and vimentin but not desmin. For an analysis of stem cells, two-color flow cytometry of isolated cells stained with Hoechst 33342 demonstrated that 0.5% of VICs were side population cells; none stained for SMA. Upon culture, sorted side population cells generated approximately 85% SMA-positive cells, indicating that some myofibroblasts originate from a rare population with stem cell characteristics. Plating cells on rigid collagen substrates enabled the formation of myofibroblasts after 5 days in culture, which was completely blocked by culture of cells on compliant collagen substrates. Exogenous tensile force also significantly increased SMA expression in VICs. Isotope-coded affinity tags and mass spectrometry were used to identify differentially expressed proteins in myofibroblast differentiation of VICs. Of the nine proteins that were identified, cofilin expression and phospho-cofilin were strongly increased by conditions favoring myofibroblast differentiation. Knockdown of cofilin with small-interfering RNA inhibited collagen gel contraction and reduced myofibroblast differentiation as assessed by the SMA incorporation into stress fibers. When compared with normal valves, diseased valves showed strong immunostaining for cofilin that colocalized with SMA in clustered cells. We conclude that in VICs, cofilin is a marker for myofibroblasts in vivo and in vitro that arise from a rare population of stem cells and require a rigid matrix for formation.
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PMID:Cofilin is a marker of myofibroblast differentiation in cells from porcine aortic cardiac valves. 1826 9

Inflammatory myofibroblastic tumor (IMT) and low-grade myofibroblastic sarcoma (LGMS) are intermediate- or low-grade malignant myofibroblastic neoplasms. In this study, the clinicopathologic profiles of 24 IMTs and 10 LGMS were compared with a focus on the immunohistochemical profiles of the neoplastic myofibroblasts. The primary antibodies used in this study were specific for the ultrastructural subcellular components: (1) *-smooth muscle actin (*-SMA), muscle-specific actin (MSA), calponin, and h-caldesmon for myofilaments; (2) fibronectin for fibronexus; (3) laminin for basal lamina; (4) desmin and cytokeratin for intermediate filaments. Anaplastic lymphoma kinase (ALK) alterations were examined by immunohistochemical means, with selective fluorescence in situ hybridization analysis. Histologically, IMT had inhomogeneous microscopic features with multi-component and multi-patterned architecture, whereas LGMS tended to be more uniform in appearance with a higher cellularity, more prominent nuclear hyperchromasia, and a more widely infiltrative growth pattern than IMT. Immunohistochemically, firstly, more than 90% of the cases of both IMT and LGMS expressed calponin, *-SMA, MSA, and fibronectin, almost all with a high expression level, and no cases were positive for h-caldesmon. Secondly, 33.3% (7/21) of IMTs and 40% (4/10) of LGMS were positive for desmin with a low expression level. The positive percentage for laminin was 81.8% (18/22) in IMT, but was 42.9% (3/7) in LGMS with a low expression level. Thirdly, 13.6% (3/22) of IMTs were positive for cytokeratin, but no expression was found in LGMS. ALK staining was found in 40.9% (9/22) of IMTs, and the presence of ALK gene rearrangements was confirmed by fluorescence in situ hybridization in 5 of 6 IMTs examined. However, neither ALK gene rearrangements nor ALK protein labeling was detected in LGMS (0/9). In summary, IMT and LGMS are both composed of cells displaying well-developed myofibroblastic differentiation, which frequently and extensively express actin-associated proteins (*-SMA, MSA, and calponin) and fibronectin, consistent with the ultrastructure markers (myofilaments and fibronectin fibrils). Laminin expression does not exclude a diagnosis of myofibroblastic neoplasms. ALK and cytokeratin, when positive, can be helpful in differentiating IMT from LGMS. LGMS is not a member of the family of ALK-positive tumors.
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PMID:Inflammatory myofibroblastic tumor and low-grade myofibroblastic sarcoma: a comparative study of clinicopathologic features and further observations on the immunohistochemical profile of myofibroblasts. 1840 Feb 54

Thrombospondin (TSP)-1, a potent angiogenesis inhibitor, has been shown to exert different biological functions on various cell types. Here, we investigate the role of TSP-1 in tumor-stroma reaction, which is mainly characterized by fibroblast activation to create a permissive microenvironment for tumor progression. Immunohistochemistry examinations in the human surgical specimens have shown that a downregulation of TSP-1 during the progression of cervical carcinogenesis was accompanied by an emergence in the upregulation of stroma markers, alpha-smooth muscle actin (alpha-SMA) and desmin. Transfection of SiHa cervical cancer cells with a plasmid expressing the TSP-1 protein exhibited antiangiogenic activity in vitro and resulted in reduced tumor growth in severe combined immunodeficiency (SCID) mice, which was accompanied by a decrease in tumor vascularization and lower expressions of alpha-SMA and desmin than those in the vector controls. Transfection with TSP-1 and purified TSP-1 added to NIH3T3 cells did not alter the protein levels of alpha-SMA and desmin but significantly inhibited matrix metalloprotease-2 activity. Transforming growth factor-beta (TGF-beta), a major factor in the activation of fibroblasts, increased alpha-SMA and desmin expression and the ability of cell migration and invasion in NIH3T3 cells. The increased migration ability and the invasive ability into tumor cluster of TGF-beta-treated NIH3T3 cells were dose dependently inhibited by TSP-1. In contrast, ectopic TSP-1 expression in SiHa cells has little effect on the invasive ability of the NIH3T3 cells. Together, our findings demonstrate a novel role of TSP-1 to inhibit tumor-stroma reaction that could be attributed to the blockage of activated fibroblasts from invading cancer cells.
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PMID:A novel role of thrombospondin-1 in cervical carcinogenesis: inhibit stroma reaction by inhibiting activated fibroblasts from invading cancer. 1841 67


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