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 expression and cellular distribution of desmin, alpha-smooth muscle actin (A-SMA) and cytokeratin no. 8 (CK-8) and no. 18 (CK-18) in normal adult, neonatal and fetal rat liver were examined immunohistochemically on cryostat sections. At days 14 and 15 of gestation, nonhematopoietic cells in embryonic liver were strongly desmin-positive, and some of the cells, mainly located in the periphery, were also stained with anti-A-SMA. Desmin immunoreactivity gradually decreased from day 16 of gestation. A close association of desmin-positive cell processes with hematopoietic cells was observed during fetal and early neonatal development. From day 16 of gestation the pre-hepatocytes became desmin-negative, remained CK-8 and CK-18 positive. Desmin-expressing cells were numerous in the liver from the embryonic period to the neonatal age. However, their absolute number per unit area, as well as their number relative to hepatocytes, decreased with age. We suggest that desmin-positive cells in embryonic liver may act as stromal cells in the hepatic hematopoietic microenvironment and support hepatocyte development.
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PMID:Desmin expressing nonhematopoietic liver cells during rat liver development: an immunohistochemical and morphometric study. 857 47

Three cases of intrahepatic biliary cystadenoma with mesenchymal stroma and one case of biliary cystadenocarcinoma are presented. Their immunohistochemical features and the surgical treatment are discussed together with a brief review of the literature. The benign cystadenomas stained positive for cytokeratin and CA 19-9 in the epithelium of the cyst wall. Mesenchymal stromal cells were strongly positive for a-SMA and moderately positive for desmin. The epithelium of the cystadenocarcinoma, however, was positive only for cytokeratin and the stroma only for a-SMA. Our findings indicate that biliary cystadenomas seem to be of primitive hepatobiliary origin. Furthermore, the malignant variant cystadenocarcinoma may loose its immunoreactivity for CA 19-9 and desmin.
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PMID:A case report of biliary cystadenoma and cystadenocarcinoma. 949 93

Hemangiopericytoma (HPC) is an uncommon vascular neoplasm thought to be derived from pericytes. Prediction of patient outcome is difficult based what is currently known about these tumors and histological parameters alone. We compiled 27 cases of HPC and evaluated the spectrum of histological features to investigate whether there was any correlation between histology, immunostaining, prognostic markers, and patient outcome. The following parameters were evaluated: vasculature, histological pattern (solid, myxoid, trabecular, alveolar), degree of cellular pleomorphism, necrosis, mitoses, and giant cell content. Immunohistochemistry was performed to determine the reactivity for CD 31, CD34, vimentin, actin, cytokeratin, S100, actin, and SMA. Proliferative rate was analyzed using antibodies to PCNA and MIB1. Patient's age ranged from 8 months to 75 years (mean, 35; median, 31). Twenty of 27 cases were located in the extremities. The tumors were grossly described as lobulated and well circumscribed (n=12) and nonencapsulated (n=15). By histology, the characteristic ramifying or staghorn vasculature pattern was seen in all cases. A solid histological pattern was mixed with an alveolar pattern in three cases, trabecular pattern in six cases, and myxoid pattern in two cases. Tumor cells were uniform, polygonal to spindle-shaped, often with vesicular nuclei. Tumor giant cells were present in 9 of 27 cases; necrosis, in 11 of 27. Mitoses ranged from 0 to 14 per 10 high-power fields (HPF). Cellular pleomorphism was 1+ in nine cases, 2+ in 12 cases, and 3+ in six cases. Immunohistochemistry showed reactivity for CD34 and vimentin in all cases. Actin was focally positive in one case, and SMA was focally positive in another. CD 31, cytokeratin, and S100 were uniformly nonreactive. Proliferative index measured by PCNA and MIBI ranged between less than 1% and 40% of tumor cells. Follow-up was available in 22 cases and ranged from 1 year to 15 years. Seven patients had metastases, and two recurred locally. Thirteen patients had no evidence of disease at last checkup. Parameters associated with recurrences or metastases include a trabecular pattern, the presence of necrosis, mitoses, vascular invasion, and cellular pleomorphism. Features associated with an aggressive biological behavior can be identified histologically. There was some, but not total, correlation between proliferative markers and tumor aggressiveness.
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PMID:The histological spectrum of hemangiopericytoma: application of immunohistochemical analysis including proliferative markers to facilitate diagnosis and predict prognosis. 963 86

Rhabdoid tumor is a well-accepted clincopathologic entity among childhood renal neoplasms; similar tumors have been described in extrarenal locations. We present the clinicopathologic profile and the immunohistochemical features of a series of soft tissue rhabdoid tumors. Twenty-eight cases coded as extrarenal rhabdoid tumor (ERRT), RT, possible ERRT, and "large cell sarcoma" were retrieved from the Armed Forces Institute of Pathology soft tissue registry. The tumors were reclassified according to strict criteria by light microscopy, clinical information, immunohistochemistry, and, in some cases, electron microscopy. Soft tissue rhabdoid tumor (STRT) was defined as (1) a tumor composed of noncohesive single cells, clusters, or sheets of large tumor cells with abundant glassy eosinophilic cytoplasm, an eccentric vesicular nucleus, and an extremely large nucleolus; (2) positivity for vimentin and/or cytokeratin or other epithelial markers by immunostaining; and (3) exclusion of other tumor types with rhabdoid inclusions (melanoma, other sarcomas, carcinoma). Eighteen cases met our criteria for soft tissue rhabdoid tumors. The median patient age was 13 years (range, 6 months to 56 years). Ninety-four percent of STRT cases were positive for vimentin and 59% for pan-cytokeratin. Sixty-three percent and 60% were positive for CAM 5.2 and EMA, respectively. Seventy-nine percent stained for at least one epithelial marker; 76% stained for both vimentin and epithelial markers simultaneously. Forty-two percent stained for MSA, and 14% for CEA and SMA. CD99, synaptophysin, CD57 (Leu-7), NSE, and focal S100 protein were identified in 75%, 66%, 56%, 54%, and 31% of the STRT cases, respectively. All STRT cases examined were negative for HMB-45, chromogranin, BER-EP4, desmin, myoglobin, CD34, and GFAP. Follow-up examination in 61% of the STRT patients revealed that 64% of patients died of disease within a median follow-up interval of 19 months (range, 4 months to 5 years); 82% had metastases to lung, lymph nodes, or liver; 22% had local recurrences before metastasis; and 18% were alive without known disease status (median, 5.5 years). Soft tissue rhabdoid tumor is a highly aggressive sarcoma, predominantly of childhood. Besides having nearly consistent coexpression of vimentin and epithelial markers, STRTs show positivity for multiple neural/neuroectodermal markers that overlap with those of primitive neuroectodermal tumor.
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PMID:Extrarenal rhabdoid tumors of soft tissue: a clinicopathologic and immunohistochemical study of 18 cases. 993 May 72

Malignant rhabdoid tumor (MRT), described for the first time in 1978 in the kidney, has rarely been reported in other organs including the brain and has involved adults in only 3 cases. We described a case of MRT in a 32-year-old woman who presented with severe headache, nausea and sudden onset of visual disturbance. MRI showed a well-enhanced mass at the suprasellar region. Subtotal removal of the tumor was performed. However, tumor regrowth occurred after the operation (doubling time, 8.36 days) and spinal dissemination was detected. Therefore, chemotherapy and radiotherapy were administered focusing on the suprasellar lesion and the spinal cord. Pathologically, light micrographs showed rhabdoid cells with large, round, single or double nuclei with one prominent nucleolus and eosinophilic cytoplasmic inclusions. Electron micrographs were made of typical rhabdoid cells displaying bundles of intermediate filaments within the perikaryon. In immunohistochemical studies, EMA, vimentin, cytokeratin and SMA were positive. Pathological findings were consistent with those of MRT. Optimal treatment for this tumor has not been established. Our case may be useful in defining treatment for MRT.
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PMID:[Suprasellar primary malignant rhabdoid tumor in an adult: a case report]. 1076 34

A 74-year-old woman complained of a small nodule in the outer lower quadrant of her left breast. On physical examination, a 0.9 x 0.8 cm, round-shaped and firmly elastic nodule was palpated. Excisional biopsy was performed. Histologically, the tumor was separated into, with a bicellular pattern, containing both numerous glandular structures and numerous spindle-shaped cells. Immunohistochemical staining for EMA and cytokeratin showed strongly positive immunoreactivity for epithelial cells. Staining for &aipha;-SMA showed strongly positive immunoreactivity for myoepithelial components. Staining for keratin and S-100 protein showed weakly positive immunoreactivity for myoepithelial cells. Microscopically, the tumor was diagnosed as adenomyoepithelioma of the breast. Immunohistochemical examination is needed to distinguish epithelial cell proliferation from myoepithelial cell proliferation. Immunohistochemical examination using antibodies against EMA, alpha-SMA, Keratin, cytokeratin and S-100 protein, is indispensable.
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PMID:Adenomyoepithelioma of the Breast: A Case Reprot and a Review of Literature. 1109 56

There is much debate over the origins of fibroblast-type cells that accumulate in interstitial fibrosis. A controversial hypothesis, supported by data from animal and cell-culture studies, is that fibroblast-type cells can derive from tubular epithelial cells by a process of epithelial-mesenchymal transdifferentiation. However, to date, no evidence supports this postulate in human glomerulonephritis. This study sought to provide evidence that tubular epithelial cells can undergo phenotypic change toward a fibroblast-like cell in human glomerulonephritis. One hundred twenty-seven open renal biopsy specimens from patients with minimal change disease (MCD), immunoglobulin A (IgA) nephropathy, and rapidly progressive glomerulonephritis (RPGN) were examined for tubular phenotypic change by two-color immunohistochemistry using the criteria of de novo expression of alpha-smooth muscle actin (alpha-SMA), a myofibroblast marker; loss of the epithelial marker cytokeratin; and collagen production. In normal human kidney and MCD, tubular epithelial cells expressed cytokeratin with no evidence of alpha-SMA staining. However, in 36 of 90 cases of IgA nephropathy and 9 of 18 cases of RPGN, small numbers of tubular epithelial cells in areas of fibrosis showed de novo alpha-SMA expression, accounting for 0.4% +/- 0.2% (IgA nephropathy) and 3.8% +/- 1.5% (RPGN) of cortical tubules. An intermediate stage of phenotypic change was observed in some cuboidal epithelial cells that expressed both cytokeratin and alpha-SMA. Tubules containing alpha-SMA-positive (alpha-SMA(+)) cells also stained for collagen types I and III, suggesting that tubular cells undergoing phenotypic change have an active role in the fibrotic process. There also was a marked increase in transforming growth factor-beta1 (TGF-beta1) tubular expression in areas with interstitial fibrosis, including tubules with phenotypic change. There was a highly significant correlation between tubular alpha-SMA expression and interstitial fibrosis, interstitial alpha-SMA(+) myofibroblast accumulation, deposition of collagen types I and III, tubular TGF-beta1 expression, and renal dysfunction. In conclusion, this study provides evidence that tubular epithelial cells can undergo phenotypic change toward a myofibroblast-like phenotype on the basis of de novo alpha-SMA expression, loss of cytokeratin, and de novo collagen staining. These data, although not conclusive, provide the first support for the hypothesis that transdifferentiation of tubular epithelial cells has a role in progressive renal fibrosis in human glomerulonephritis.
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PMID:Tubular phenotypic change in progressive tubulointerstitial fibrosis in human glomerulonephritis. 1157 79

The atypical teratoid / rhabdoid tumour is a rare type of tumours of central nervous system appearing usually under 2 years of age, bearing a rather bad prognosis and it may cause serious differential diagnostic problem. The tumour is characterized histologically by the presence of the rhabdoid cells, immunohistochemically by vimentin, SMA, EMA positivity, the frequent presence of cytokeratin, GFAP positivity, but germ cell markers: AFP, hCG negativity, cytogenetically by aberrations of chromosome 22. The case of a one and half month old female infant is presented, who died 8 months after the appearance of the first symptoms. The diagnostic possibilities and the unsolved problem of the therapy are discussed.
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PMID:[A case of central nervous system atypical teratoid / rhabdoid tumor] 1205 Jul 40

Basaloid squamous cell carcinoma (BSCC) is a recently recognized high-grade tumor with a propensity for nodal as well as systemic metastasis and can arise from different anatomic locations. The differential diagnosis includes adenoid cystic carcinoma, small cell neuroendocrine carcinoma and squamous cell carcinoma. Monoclonal antibodies reactive with cytokeratin (34betaE12, AE3, pancytokeratin), as well as other cellular antigens (vimentin [VIM]; synaptophysin [SYNF]; chromogranin A [ChA]; neuron-specific enolase [NSE]; S-100, desmin, smooth-muscle actin [SMA]), were used in an immunoperoxidase method with paraffin-embedded tissue to phenotypically characterize a case with features of BSCC arising in the maxillary sinus. Neoplastic cells reacted with the high-molecular-weight cytokeratin antibody 34betaE12, as well as with other antikeratin antibodies, but failed to react with the antibodies VIM, desmin and SMA and showed variable immunoreactivity for NSE, SYNF and S-100. The staining pattern for NSE was diffuse and intense and reactivity for ChA was inconsistent.
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PMID:Basaloid squamous cell carcinoma of the maxilla: a case report and immunohistochemical analysis. 1212 1


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