Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0268596 (EMA)
 2,520 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The previous biochemical evidence had suggested that glutaric aciduria type II (GA II) is due to deficient dehydrogenation of multiple short-chain acyl coenzyme A's (CoA's), bu the precise biochemical mechanism underlying this disease was unknown. We investigated substrate oxidation and in vitro activities of isovaleryl CoA- and butyryl CoA dehydrogenases as well as that of electron-transferring flavoprotein (ETF) in cultured skin fibroblasts from a patient with GA II. GA II cells have a markedly decreased ability to oxidize [1-14C]butyrate, [2-14C]lysine, and [2,14C]leucine (3, 9, and 9% of control values, respectively). Mitochondrial isovaleryl CoA- and butyryl CoA dehydrogenase activities in GA II cells were determined using a tritium release assay with [2,3-3H] acyl-CoA's as substrate. When an artificial electron acceptor, phenazine methosulfate (PMS) was not added in the assay media, these activities were 108 and 113% of controls, respectively. This represents the normal abilities of the dehydrogenases in GA II cells to bind the substrate and to catalyze tritium exchange between the bound substrate and solvent. When PMS was added to the assay mixture, these activities were 88 and 70% of control values, respectively, indicating that these enzymes can both dehydrogenate their substrates normally and then transfer electrons to an acceptor (PMS). ETF activity in mitochondrial sonic supernatants from GA II cells, as assessed by a newly devised method, was 159% of control values. These observations suggest that the acyl CoA dehydrogenases themselves and ETF are not defective in GA II. Therefore, the deficiency of another common gene product necessary for the function of all the affected acyl CoA dehydrogenases must be sought to explain the etiology of GA II.
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PMID:Glutaric aciduria type II: in vitro studies on substrate oxidation, acyl-CoA dehydrogenases, and electron-transferring flavoprotein in cultured skin fibroblasts. 720 50

The glandular peripheral nerve sheath tumor is a rare variant of nerve sheath neoplasms in which the focally occurring glands are lined by cells showing divergent differentiation. The vast majority of the reported nerve sheath tumors harboring these glands have been malignant. We herein present a case of benign glandular peripheral nerve sheath tumor in a 43-year-old woman who had no evidence of von Recklinghausen's disease. Histologically, the tumor is composed of spindle cell component and collections of glandular component. The glandular component occupied the central two-thirds of the lesion and was lined by a single layer of nonciliated cuboidal or columnar cells. No mitotic figures were recognized in the spindle cell area. This spindle cell area had neurofibroma-like features rather than schwannoma. Many of the spindle cells had positive reaction products for S-100 protein. The glandular lining epithelium were positive for cytokeratins (CAM 5.2, AE1/AE3, PKK1) and EMA. Some epithelial cells were immunoreactive for CEA, chromogranin, somatostatin and Leu-7. These immunohistochemical findings support the neuroendocrine differentiation of the epithelial element from the schwannian component.
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PMID:Benign glandular peripheral nerve sheath tumor. A case report. 752 35

Eighty-three kidneys from autopsy cases, all more than 60 years of age, were used in the present studies. Three millimeter-thick step slices from all kidneys were embedded in paraffin, and serial sections from all blocks used for the immunohistochemical demonstration of Leu M1 (leukocyte membrane antigen) and LTA (Lotus tetragonolobus agglutinin) in cells of proximal convoluted tubular origin, and PNA (peanut agglutinin) and EMA (epithelial membrane antigen) in cells of distal convoluted tubular origin. The ABC staining method was used in all cases. A total of 65 renal cell adenomas found in 31 of the 83 kidneys consisted of 40 papillary, 20 tubular and 5 solid type lesions. The sizes of these renal cell adenomas were from 0.6 to 5 mm in diameter and compression of neighboring tissues was characteristic. Papillary renal cell adenomas were positive in their cytoplasms for Leu M1 and LTA in 7 cases and at their cell membranes for PNA and EMA in 33 cases. The respective figures for tubular renal cell adenomas were 6 cases for Leu M1 and LTA and 14 cases for PNA and EMA. All solid renal cell adenomas were positive in their cytoplasms for PNA and EMA. The immunohistochemical results thus indicated 13 of 65 lesions to have a proximal convoluted tubular cell origin and 52 to be possibly derived from distal convoluted tubules or collecting ducts. A role for metaplasia, however, could not be ruled out.
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PMID:Antigen immunohistochemistry of renal cell adenomas in autopsy cases: relevance to histogenesis. 753 36

When graft-versus-host (GVH) disease affects the liver, it is characteristically the bile ducts which are involved, infiltrated by lymphocytes. To characterize this process further, and to determine whether there were any antigenic changes in the bile ducts, we stained 9 liver biopsies involved by GVH disease, 10 non-GVH biopsies that had a prominent portal lymphocytic component, and 8 biopsies taken incidentally at surgery for noninflammatory liver disease with epithelial membrane antigen, AE-3, AE-1, a keratin cocktail, keratin 19, CD45RO (UCHL-1), CD43 (Leu-22), CD20 (L26), vimentin, and LN-3. The infiltrating lymphocytes were T cells (CD45RO+, CD43+, CD20-) which variably expressed LN-3. The bile ducts were positive for the keratin cocktail, AE-1, AE-3, and keratin-19, but only occasionally positive for EMA and LN-3. There was no significant difference in the staining patterns of either the bile duct cells or lymphocytes between the three groups. With the antibodies that we used, there does not appear to be a significant difference in the antigenic phenotype of the bile ducts in GVH as compared to normal or reactive livers.
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PMID:Phenotype of bile ducts and infiltrating lymphocytes in graft-versus-host disease. 768 95

Techniques of production of monoclonal antibodies (MoAb) have provided powerful tools to study biological lung cancer behavior. Immunochemistry is more sensitive than conventional light microscopy examination to detect tumour cells in sputum or pleural effusion, or small cell lung cancer metastases in bone marrow. Immunochemistry is also helpful for the differential diagnosis of carcinoma versus lymphoma or sarcoma, using antibodies directed against antigens such as cytokeratins, vimentin, EMA, LCA, SP100, CEA. In lung cancer, immunochemistry may detect neuroendocrine differentiation, or help to distinguish metastatic carcinoma from primary lung cancer. A positive immunostaining with CEA, Leu-M1, SP1, B72-3 supports the diagnosis of pleural metastatic adenocarcinoma versus mesothelioma. Immunoscintigraphy is a non invasive imaging technique which allows local and distant disease evaluation and could replace in the future the present staging work up. To evaluate the potential therapeutic efficacy of MoAbs in Lung cancer, phase I studies have been performed. Therapeutic effect is based on: 1) indirect cytotoxicity (cells are killed by ADCC or K cells) or direct cytotoxicity (MoAb are carriers of toxins, radioisotopes or drugs). 2) Immune response modulation by anti-idiotypic Ab. 3) Interferences with growth factors. Results of most of phase I trials are disappointing. Improvement of MoAb selectivity, improvement of conjugates stability, reduction of humoral response to MoAb, enhanced tumour localisation, and reduction of nonspecific captation should lead to a better efficacy.
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PMID:[Monoclonal antibodies and bronchial cancer]. 770 64

A case of malignant peritoneal mesothelioma mimicking mesenteric inflammatory disease (MID) is presented. The patient had mesenteric and omental lesions characterized at biopsy by extensive fibrosis of fat tissue with mild to moderate inflammation. One year later, post-mortem examination revealed a well-differentiated epithelial mesothelioma. Immunohistochemical stains for keratin and vimentin were diffusely positive, whereas EMA showed a membranous staining of scattered cells. CEA, Ber-EP4, B72.3 and Leu-M1 were negative. In addition, actin monoclonals decorated groups of cells pertaining to the tumoural component. Immunostains of sections from retrieved paraffin blocks of the previous biopsy showed that the bulk of the spindle-shaped and histiocytic-like cells present in the fibrous streams was strongly labeled by low-molecular-weight keratin, and coexpressed vimentin and actin. EMA showed a membranous staining of sporadic spindle and round cells. The other immunostains were invariably negative. This immunohistochemical pattern closely corresponded to the immunophenotype of the mesothelial tumour detected at autopsy and was very suggestive of myofibroblastic/submesothelial cell origin. The quantitative evaluation of silver nucleolar organizer regions (Ag-NORs) demonstrated high levels of cell proliferation in both surgical and autopsy tissue samples.
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PMID:Malignant peritoneal mesothelioma mimicking mesenteric inflammatory disease. 798 21

Microwave oven (mwo) is used to stimulate tissue fixation and to retrieve antigens damaged by fixation. Heavy metal salt solutions, water, and citric acid buffer (cab) have been suggested for this purpose. A serie of tumors treated with cab and phosphate-buffered saline (pbs) with mwo were studied immunohistochemically with 24 antibodies. Controls were treated in the same way, except for microwaving. The antibodies were directed against antigens of the following tumors: breast and prostate carcinoma, carcinoid, lymphoma and melanoma. The results showed that cab enhanced the immunoreactivity of the following antigens: estrogen receptors (AMAC), progesterone receptors (Novocastra), HMB45, vimentin, leukocyte common antigen, PCNA, p53, MIB-1 (Ki-67) and prostatic specific antigen. The antigens that did not improve their immunoreactivity, when compared with the control series were: factor VIII, keratin, Leu 22, L26, neuron-specific enolase, CEA, chromogranin, HBME-1, smooth muscle actin and EMA. Microwaving equally improved protein S100 and desmin either with cab or pbs. The only antigen that improved with pbs was actin. The results with B72.3 and NKI/C3 were poor and not reliable. In conclusion microwaving with cab enhances the immunoreactivity of the antibodies mentioned above leading to an increase in sensibility without loosing specificity.
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PMID:[Antigen retrieval by microwave oven with buffer of citric acid]. 799 28

The expression of surface antigens on human type II pneumocytes is unknown but may be important in diagnostic cytology of bronchoalveolar lavage specimens. Thus, the immunocytochemical reactivity of isolated human type II pneumocytes was determined using a panel of commercially available monoclonal antibodies (MAbs). Type II pneumocytes were isolated from fresh human lung tissue obtained from surgical specimens (four non-smokers, six heavy smokers) after enzymatic digestion with dispase and subsequent discontinuous metrizamide gradient centrifugation. MAbs OKIa; EMA; OKT9; BMA 130a, b and c; EP4; TAG 72; HEA 125; and Leu M1 were studied using the peroxidase-antiperoxidase adhesive slide assay method. In all cases, type II pneumocytes reacted positively with OKIa, BMA 130a, BMA 130b, EMA, EP4, TAG 72 and HEA 125 and negatively with OKT9, BMA 130c and Leu M1. The percentage of positively reacting type II pneumocytes was 90 for OKIa, HEA 125 and EP4; 80 for EMA; 50 for TAG 72 and BMA 130a; and 5 for BMA 130b. Human type II pneumocytes share the expression of several antigens with epithelial tumor cells. This limits the usefulness of these markers with respect to differentiating between reactive type II pneumocytes and malignant cells.
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PMID:Immunocytochemical characterization of isolated human type II pneumocytes. 804 19

In a randomized design we examined the interobserver variation in the histopathological diagnosis of adenocarcinoma of the lung and malignant mesothelioma. In three rounds, three pathologists assessed slides from 42 tumours originally diagnosed as adenocarcinomas, malignant mesotheliomas or benign lesions in the pleura. In the first round the assessments were made on haematoxylin and eosin (H & E) stained sections; in the second, on H & E sections plus sections stained with histochemical mucin stains; and in the final round, the diagnoses were made on H & E sections and sections stained with a panel of antibodies against various antigens (cytokeratin, EMA, CEA, Ber-EP4, B72.3, Leu-M1, vimentin and S-100 protein) said to be of value in the differential diagnosis. The overall interobserver agreements for the three rounds were 0.659, 0.802 and 0.817; the kappa values were 0.461, 0.681 and 0.690. It is concluded that differentiation between adenocarcinoma of the lung and malignant mesothelioma should be made on sections stained with H & E and mucin and/or immunohistochemical staining reactions, including antibodies against B72.3, Ber-EP4 and CEA.
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PMID:The histopathological diagnosis of malignant mesothelioma v. pulmonary adenocarcinoma: reproducibility of the histopathological diagnosis. 806 83

Diagnosis of mesothelioma is a difficult clinical and pathological task. The morphology of the neoplasm is extremely variable and is the major cause of the diagnostic dilemma. Malignant mesotheliomas are difficult to distinguish from benign reactive lesions of the pleura and from metastatic neoplasms, particularly adenocarcinomas and IVBAT (intravascular bronchioloalveolar tumor better referred to as sclerosing angiosarcoma or epithelioid hemangioendothelioma). Immunohistochemical studies represent a crucial diagnosis aid. A practical approach in different morphological situations is described and a routine antibody panel is proposed: the dual negativity of CEA and Leu M1 associated with thick "hairy" brush borders with EMA staining suggest malignant epithelial mesothelioma. The dual positivity of CEA and Leu M1 and the presence of thin smooth borders staining with EMA suggest adenocarcinomas. Behind a spindle-cell neoplasm the coexpression of keratin, vimentin and the presence of keratin-positive cells infiltrating underlying tissue suggest malignant desmoplastic mesothelioma.
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PMID:[Immunohistochemistry of mesotheliomas. Technique and current diagnostic contribution of immunohistochemical markers. General review]. 813 82


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