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)

A high prevalence of antibodies to cytoskeleton components (anti-CYTO) and of anti-smooth muscle (SMA) and antinuclear (ANA) antibodies has been found in inflammatory bowel disease (IBD). In particular, a significant correlation has been documented between anti-CYTO and activity of the disease in ulcerative colitis and anti-fibroblast intermediate filaments (anti-vimentin) antibodies and Crohn's disease. The antibodies are detectable in the three major immunoglobulin classes, including IgA. While titres of anti-CYTO did not exceed 1:40, ANA and SMA were of high titres (up to 1:1,280). Antibodies to epithelial cells intermediate filaments (anti-cytokeratin) were detected only occasionally. The significance of these findings in IBD is discussed.
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PMID:Antibodies to the cytoskeleton components and other autoantibodies in inflammatory bowel disease. 241 21

The histogenesis of cutaneous focal mucinosis (CFM) is controversial. Eleven cases of CFM (5F, 6M; mean age 51 years) from our routine files between 1986 and the present time have, therefore, been examined histopathologically and immunohistochemically. Histology revealed an increased number of fibroblast-like cells in early lesions, whereas they were diminished or predominantly at the margin in advanced ones. The myxomatous areas showed slight to absent reticulum formation. Similarly, elastic fibers were almost absent, and collagen fibers were fragmented and replaced by variable amounts of mucin. One specimen revealed an epithelial component within the lesion reminiscent of a poorly induced trichofolliculoma. Immunohistochemically, vimentin was consistently present and correlated with the number of fibroblast-like cells. A few (< 5%) CD34+ dermal dendritic cells (DDs) were focally seen within CFM. In contrast, FXIIIa+ DDs accounted for up to 30%. Fibroblast-like cells were negative for S-100 protein, Leu7, desmin and alpha-SMA. The epithelial component within one of our specimens seems to have been induced by CFM and is a feature also seen in (angio)-myxomas. CFM appears to be a mesenchymally derived lesion composed predominantly of fibroblasts. DDs do not form the major cell component but rather seem passively incorporated.
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PMID:Cutaneous focal mucinosis--a histopathological and immunohistochemical analysis of 11 cases. 753 54

Cytogenetic and immunohistochemical studies were performed in nine myxoid liposarcomas. The tumor karyotype was determined after short-term culture of cells in vitro. Immunohistochemical studies were performed on frozen tissue in five cases and on paraffin-embedded tissue in three cases. Chromosomal analysis demonstrated a balanced translocation t(12;16) (q13;p11) as the sole abnormality in four cases. Two cases showed an association with other abnormalities. Three tumors showed variants of the t(12;16) translocation involving other chromosomes. In all cases studied, the 12q13 breakpoint was involved in rearrangements. In the majority of cases, immunohistochemical studies demonstrated vimentin (9 of 9) and S-100 protein (8 of 9). Strong focal expression of desmin was observed in two tumors. Weak focal expression was observed in three tumors. Two tumors, which were both desmin positive, showed focal expression of MSA and alpha-SMA. Strong expression of CD36 was present in all four cases that were studied for this marker. CD34 was negative in tumor cells, but it highlighted an intricate capillary network in the tumor. Close relationship between the tumor cells and pericapillary pericytes was demonstrated with CD34 and alpha-SMA strains. The authors conclude that myxoid liposarcoma is characterized by a specific chromosomal rearrangement. Its immunohistochemical profile is wider than previously believed, including expression of muscle markers.
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PMID:Cytogenetic and immunohistochemical profile of myxoid liposarcoma. 781 37

Chondroid syringoma belongs to the group of so-called mixed tumors, like pleomorphic adenomas of the lacrimal and salivary glands. The histogenesis of this tumour is still disputed, in particular with respect to its stromal component. The distribution of cytokeratins (CKs), CEA, EMA, vimentin, S-100 protein, desmin and actin [alpha-smooth muscle actin (alpha-SMA)] was investigated by immunohistological examination of paraffin sections from a chondroid syringoma of the apocrine type. The neoplastic formations have been classified into tubuloalveolar structures, solid nests/aggregations and stromal cells of varying morphology. The inner-most cells of tubuloalveolar structures were characterized by marked expression of CKs (KL1 and MNF116), CEA and EMA, while in the outer ones there was moderate expression of vimentin, S-100 was expressed to a lesser extent and KL1, weakly but there was marked and consistent expression of MNF116. Whereas the solid nests expressed vimentin, S-100 protein, MNF116 markedly and KL1 weakly, the stromal cells were consistently positive for vimentin, S-100 protein and, focally, CKs and alpha-SMA. Anti-alpha-SMA specifically detects myoepithelial cells. In addition, the partly overlapping immunoreactivity of the intermediate filaments, membrane proteins and proteins in the different structures may indicate a common clonal origin of all neoplastic cells in chondroid syringoma.
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PMID:[Chondroid syringoma. Immunohistologic indications of myoepithelial differentiation]. 805 Sep 3

Immunohistochemical methods were used to distinguish idiopathic pulmonary fibrosis (IPF) from interstitial pneumonia associated with rheumatoid arthritis (RA lung). The subjects were six patients with IPF and seven with RA lung, in whom the pathological findings were consistent with usual interstitial pneumonia. Antibodies to vimentin (Vim), alpha-smooth muscle actin (alpha-SMA), and S-100 protein were used for immunohistochemical studies done by the streptavidin-biotin-peroxidase complex method. In fibrosis associated with RA lung, proliferation of both Vim and alpha-SMA-positive myofibroblasts was widely observed, despite pathological findings of honeycombing, usual interstitial pneumonia, and BOOP. Fibrosis in cases of IPF was found to be characterized mainly by Vim-positive fibroblasts, and on occasion was associated with hyperplasia of smooth muscle. Lung tissues from patients with acute exacerbations of RA lung, especially when associated with a BOOP pattern, had many cells positive for S-100 protein. However, such cells were generally hard to find in cases of IPE. Similar results were obtained with regard to the honeycomb pattern in both IPF and RA lung. These findings suggest that IPF and RA lung can be fairly clearly differentiated based on the proliferation of myofibroblasts and on the presence or absence of cells positive for S-100 protein.
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PMID:[Immunohistochemical study of fibrosis and S-100 protein-positive cells in idiopathic pulmonary fibrosis and in interstitial pneumonia associated with rheumatoid arthritis]. 871 85

Immunohistochemical study was carried out in patients with collagen vascular disease associated with interstitial pneumonia. The subjects were 16 patients, consisting of seven rheumatoid arthritis (RA), five dermatomyositis (DM) and four progressive systemic sclerosis (PSS), in whom the pathological findings were consistent with usual interstitial pneumonia. Immunohistochemical examinations were performed by the ABC method using antibodies to vimentin (vim), alpha-smooth muscle actin (alpha-SMA), and S-100 protein. In fibrosis associated with RA, proliferation of alpha-SMA-positive myofibroblasts was widely observed in all subjects. Myofibroblasts were present also in patients with DM and PSS, but not as notable as in those with RA. Proliferation of vim-positive fibroblasts was observed in patients with idiopathic pulmonary fibrosis (IPF). Diverse S-100 protein positive cells appeared in patients with acute exacerbations of RA, especially when associated with bronchiolitis obliterans organizing pneumonia (BOOP) pattern. S-100 protein positive cells were observed occasionally also in patients with DM and PSS, but they markedly decreased in number, compared to those with RA. They were generally hard to detect in lungs of patients with IPF. These findings suggest that interstitial pneumonia associated with collagen vascular disease can be fairly clearly differentiated from IPF each other, based on the degree of proliferation of myofibroblasts and on the presence of S-100 protein positive cells in number.
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PMID:[Immunohistochemical study of myofibroblast and S-100 protein positive cells in interstitial pneumonia associated with collagen vascular disease]. 912 18

A cell line (KMY-J) was established from a transplantable tumor (MM-KMY) derived from a spontaneous malignant meningioma arising in an aged F344 rat, and three cloned cell lines (KMY-1, KMY-2 and KMY-3) were induced from the parent KMY-J. Morphologically, KMY-J and tumors induced in syngeneic rats by KMY-J showed cell pleomorphism. All neoplastic cells in KMY-J and its tumors were immunoreactive to vimentin; occasional cells reacted to ED1 (rat macrophage/histiocyte-specific antibody) and alpha-smooth muscle actin (alpha-SMA), indicating expression of histiocytic or myofibroblastic immunophenotypes of meningioma cells. In contrast, KMY-1, KMY-2 and KMY-3 consisted of a uniform cell population differing from each other. KMY-1-induced tumors were similar histologically to meningeal fibrosarcomas. Dendritic cells seen in KMY-2 cultures gave an appearance of arachnoid trabecular cells. In KMY-3 and its tumors, large round cells and multinucleated giant cells were predominant. Cells of these cloned cell lines also reacted to vimentin, but were negative for ED1 and alpha-SMA. By the bioassay using PC12 cells and reverse transcription-polymerase chain reaction for nerve growth factor (NGF) mRNA, production of NGF was demonstrated in the parent and cloned cell lines. The present cell lines may prove useful for studying the histological features of meningeal tumors and the bioactive factors produced by meningeal cells.
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PMID:Establishment and characterization of cell lines derived from a transplantable rat malignant meningioma: morphological heterogeneity and production of nerve growth factor. 914 84

We report on eight cases of a distinct variant of cutaneous schwannoma characterized by prominent Verocay body formation (75-100% of the tumor bulk) that may cause considerable diagnostic difficulties. Like ordinary cutaneous schwannomas, these lesions preferred the head and neck region of young adults without sexual predilection and were clinically interpreted as cyst, basal cell carcinoma, or nevus. Histological examination revealed well-circumscribed nodules. Three of them consisted exclusively of nodular or ribbon-like Verocay bodies. A variable admixture of Antoni A or B type of differentiation (< 25%) was seen in five other cases. The following patterns were seen: fascicular spindle-shaped, onion-like epithelioid, myxoid-hypocellular, and degenerated ("ancient") with prominent fibrosis/hyalinosis and occasional bizarre giant cells. Immunohistochemically, the lesions were positive for S-100 protein (and vimentin) but negative for a broad panel of neurogenic and intermediate filament markers. The capsule showed focal labeling for EMA and--when it was markedly thickened--also for SMA. Labeling with E9, an anti-metallothionein marker indicative of cell activity, was negative, underscoring the slow growth potential of these lesions. No recurrence was seen in the six patients with follow-up information. The differential diagnosis includes other lesions with prominent palisading. (Amianthoid) myofibroblastoma and palisading leiomyoma are consistently positive for SMA and desmin, respectively. Palisading cutaneous fibrous histiocytoma and myofibroblastic dermatofibroma are variably positive for Factor XIIIa, SMA, and E9 and/or NK1C3 (CD57). Palisaded encapsulated neuromas are primarilly differentiated by the presence of nerve fibers with myelin sheaths.
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PMID:Verocay body--prominent cutaneous schwannoma. 1002 39

A 6-month-old girl was admitted to another hospital because of consciousness disturbance, preceded by 2 weeks of decreased activity and vomiting. She was referred to our hospital after ventricular drainage had been instituted for hydrocephalus and the tumor in the pineal region. The patient was noted to have conjugate upward gaze palsy and papilledema. CT scan and MRI revealed a large tumor in the pineal region with tumoral hemorrhage and a small mass in the right frontal lobe. At surgery, the pineal region tumor was removed subtotally. Histological examination showed the tumor to be composed of sheets of large polyhedra or round cells with an eccentric round nuclei, prominent nucleoli, and cytoplasmic inclusions. Immunohistochemical studies were positive for GFAP, vimentin, S-100, CK, EMA, and SMA, but negative for AFP, HCG, PLAP, and CEA. Following surgery, she received three 5-day cycles of chemotherapy, consisting of intravenous administration of cisplatin 20 mg/m2/day and etoposide 60mg/m2/day. After these therapies, MRI showed a decrease in the area of high intensity in the pineal region, but almost no change in the right frontal mass lesion. Follow-up radiological examination showed that the tumor had grown rapidly one month after chemotherapy and the patient died 5 months after her first hospitalization. Malignant rhabdoid tumor of the CNS is rare and remarkably malignant. This tumor should be treated using multidisciplinary management with surgery, intensive chemotherapy, and radiotherapy depending on the patient's age.
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PMID:[A case of malignant rhabdoid tumor in the pineal region in early infancy]. 930 Apr 49

In this study, we present a new method to obtain pure, viable, freshly isolated hepatic stellate cells. Stellate cells were purified by cell sorting using their high side scatter (SSC) of incident light. Purity of the cells was established by light and transmission electron microscopy (TEM). Starting from stellate cells that were 50% to 70% enriched by centrifugation in 11% Nycodenz, the cell purity after sorting was found to be 96.6% +/- 2.9%. Viability of the sorted cells was 90.8% +/- 2.2% as measured by the Trypan blue exclusion test and was confirmed by cell culturing. Per hour of sorting, 1.4 +/- 0.4 million stellate cells were obtained. Sorting runs of up to 4 hours were practically feasible, resulting in yields of 5 to 6 million cells per rat liver. Cells attached to plastic substratum within 24 hours. Subsequently, they spread and underwent spontaneous transition into myofibroblast-like cells. The purity of sorted cells was documented by reverse-transcriptase polymerase chain reaction (RT-PCR) experiments using specific primer pairs for messenger RNA (mRNA) species that were only present in parenchymal (preproalbumin), endothelial (endothelial cell nitric oxide synthase [eNOS]), stellate (desmin), or Kupffer cells (77- to 88-kd fucose receptor). Contaminating mRNA species were absent in sorted stellate cells. Next, we examined freshly sorted stellate cells by Western blotting to confirm the presence of relevant cytoskeletal proteins. Cells were positive for vimentin, desmin, and glial fibrillary acidic protein (GFAP), but negative for alpha-smooth muscle actin (alpha-SMA). Sorted and cultured cells were immunophenotyped for the presence of collagen types I, III, and IV, laminin, and the cytoskeletal proteins, alpha-SMA, desmin, vimentin, and GFAP. At 90 hours in culture, cells expressed all the investigated extracellular matrix proteins. Desmin was present in 82% +/- 1%, vimentin in 96% +/- 2.5%, and GFAP in 91% +/- 4.5% of cells. Alpha-SMA was present in 91% +/- 2% of cultured cells. We conclude that cell sorting based on SSC of incident light is a convenient method to obtain virtually pure stellate cells that can be used for direct analysis or for culturing. Although the yields obtained with this method are lower than with standard methods, and additional equipment is required, SSC-activated sorting offers the possibility of very pure cells when essential for analyses based on sensitive detection methods such as RT-PCR.
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PMID:Purification of rat hepatic stellate cells by side scatter-activated cell sorting. 946 62


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