Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: EC:3.4.24.11 (CD10)
 9,792 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

In this study, we identified lysine residues in the fibrinogen Aalpha chain that serve as substrates during transglutaminase (TG)-mediated cross-linking of plasminogen activator inhibitor 2 (PAI-2). Comparisons were made with alpha(2)-antiplasmin (alpha(2)-AP), which is known to cross-link to lysine 303 of the Aalpha chain. A 30-residue peptide containing Lys-303 specifically competed with fibrinogen for cross-linking to alpha(2)-AP but not for cross-linking to PAI-2. Further evidence that PAI-2 did not cross-link via Lys-303 was the cross-linking of PAI-2 to I-9 and des-alphaC fibrinogens, which lack 100 and 390 amino acids from the C terminus of the Aalpha chain, respectively. PAI-2 or alpha(2)-AP was cross-linked to fibrinogen and digested with trypsin or endopeptidase Glu-C, and the resulting peptides analyzed by mass spectrometry. Peptides detected were consistent with tissue TG (tTG)-mediated cross-linking of PAI-2 to lysines 148, 176, 183, 457 and factor XIIIa-mediated cross-linking of PAI-2 to lysines 148, 230, and 413 in the Aalpha chain. alpha(2)-AP was cross-linked only to lysine 303. Cross-linking of PAI-2 to fibrinogen did not compete with alpha(2)-AP, and the two proteins utilized different lysines in the Aalpha chain. Therefore, PAI-2 and alpha(2)-AP can cross-link simultaneously to the alpha polymers of a fibrin clot and promote resistance to lysis.
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PMID:Cross-linking of plasminogen activator inhibitor 2 and alpha 2-antiplasmin to fibrin(ogen). 1081 85

Distinguishing hepatocellular carcinoma (HCC) from cholangiocarcinoma (CC) and metastatic adenocarcinoma (MA) involving the liver can be problematic, often requiring the use of immunohistochemistry to facilitate diagnosis. Hep Par 1, a monoclonal antibody with expression confined primarily to benign and malignant hepatocytes, has recently become commercially available. We evaluated Hep Par 1 along with other immunohistochemical markers used to differentiate HCC, CC, and MA, including AE1/AE3, CAM 5.2, B72.3, monoclonal carcinoembryonic antigen (mCEA), polyclonal CEA (pCEA), alpha-fetoprotein (AFP), factor XIIIa, inhibin, CD10, villin, MOC-31, cytokeratin (CK) 7, CK 19, and CK 20, to determine the markers most useful in differentiating these entities. Forty-two cases of HCC, 9 cases of CC, and 56 cases of MA (24 colon, 15 pancreas, 8 ovary, 5 breast, and 4 stomach) were studied. Hep Par 1 was sensitive and specific for HCC, with 38 of 42 (90%) cases staining positively, whereas reactivity was observed in only 8 of 56 (14%) MAs and 0 of 9 CCs. Though limited somewhat by poor sensitivity, a bile canalicular pattern of staining with pCEA, CD10, and villin was specific for HCC and was not observed in the other tumors. Lack of mCEA and MOC-31 immunoreactivity was also characteristic of HCCs. CK 19 positivity favored CC over HCC, but was not useful in differentiating CC from MA. Expression of AFP, although observed in only about one third of the cases, favored HCC over CC and MA. CK 7 and CK 20 were also useful in this differential diagnosis, particularly when dealing with MA of colonic origin. AE1/AE3, CAM 5.2, B72.3, inhibin, and factor XIIIa were noncontributory in differentiating these entities.
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PMID:Comparative immunohistochemical profile of hepatocellular carcinoma, cholangiocarcinoma, and metastatic adenocarcinoma. 1251 85

We describe two elderly patients with follicular lymphoma (FL) involving the skin and superficial soft tissues, with a striking proliferation of follicular dendritic cells (FDC). In addition, one patient had bone marrow involvement by FL. Histopathologically, the most remarkable feature in both cases seen at low magnification was a striking pallor of the constituent cells, which were arranged in fascicles, whorls, and round islands. The majority of the cells had the typical cytologic features of FDCs. They were intimately intermingled with centroblasts and centrocytes. A large amount of the clear cytoplasm and the pale nuclei of FDCs, which predominated in the tumors, caused the striking overall pallor of the lesions. Small reactive lymphocytes were scattered between the fascicles. A vague follicular growth pattern was seen only focally. The mantle zones were markedly reduced or absent so that the follicles were seen lying unseparated. The close intermixture of the FDCs and the germinal center cells was responsible for the FDCs appearing to be decorated with B-associated marker, and the germinal center cells seemed to be stained to some degree with FDC-markers. The tumor bulk demonstrated a diffuse and strong reaction with CD10, CD20, CD21, CD35, and stained weakly with CD79a. Fascin and CD23 showed only a weak and focal staining pattern. Bcl-2 decorated large centroblasts and small reactive T-cells. The tumor bulk was negative for actin, EMA, cytokeratins, vimentin, desmin, and factor XIIIa. The proliferative index was rather low; MIB-1 mainly decorated large centroblasts. No monoclonal rearrangement of IgH genes was detected. Epstein-Barr virus was not identified. Electron microscopy revealed typical features of FDCs intermingled with germinal center cells. Such cases may represent a diagnostic pitfall, as FDC overgrowth can mask FL and give the neoplasm the appearance of FDC sarcoma/tumor. We believe that, in both cases, the FDC proliferation had a reactive character.
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PMID:Follicular lymphoma of the skin and superficial soft tissues associated with a prominent follicular dendritic cell proliferation: an unusual pattern which may represent a diagnostic pitfall. 1546 4

To evaluate and compare the immunophenotype of endocervical and endometrial stromal cells and to asses its potential application in tumor localization. Paraffin sections of benign endocervix (n = 24), benign endometrium (n = 33), endocervical adenocarcinoma (n = 9), endometrial carcinoma (n = 13), and endometrial hyperplasia (n = 16) were stained with antibodies to CD10, Wilms Tumor-1, CD34, smooth muscle actin, and factor XIIIa by immunohistochemistry. In 16 cases, lower uterine segment was also available. Immunoreactivity of stromal cells was recorded as positive (>/=50% staining), focally positive (>/=5%-<50%) or negative (<5%). Endocervical stromal cells (ECSC) in either benign or malignant cervical epithelial lesions were predominantly CD34/CD10 (CD34 dominant immunophenotype). Endometrial stromal cells (EMSCs) in either benign or malignant epithelial lesions were primarily CD34/CD10 (CD10 dominant immunophenotype). Expression of Wilms Tumor-1 was decreased in EMSC of the EMCA when compared to their counterpart in endometrial hyperplasia. There was no differential expression of smooth muscle actin and factor XIIIa identified between ECSC and EMSC. The immunophenotypes of the ECSC and EMSC overlapped in the lower uterine segment. The functional status of the endometrium had no effect on the immunoprofile. The pattern of CD34 and CD10 immunostaining in stromal cells might be helpful in determining tumor involvement in uterine and cervical sites.
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PMID:Immunoprofile of endocervical and endometrial stromal cells and its potential application in localization of tumor involvement. 1719 1

Neurothekeoma is a rare benign dermal lesion that is commonly seen in children and teenagers. Despite its name, the true nature of this lesion is uncertain and controversial, particularly after the emergence of the cellular (spindle/epithelioid) variant. We describe the histological and immunohistochemical findings of a right thigh skin lesion in an 11-year-old girl. It consists of a dermal ill-defined plexiform mass composed of nests and fascicles of spindle cells with pale eosinophilic cytoplasm that lie within a sclerotic stroma. The immunohistochemistry shows diffuse reactivity to CD68, matrix metalloproteinase-II, CD10 and PGP9.5 with focal reactivity to CD57 and CD34. The lesion is negative for S100, factor XIIIa, smooth muscle markers and melanocytic markers. The features are compatible with a cellular variant of neurothekeoma with plexiform pattern that also exhibits an unusual pattern of fibrohistiocytic phenotype. Although such a lesion is benign, it has a wide but important differential diagnoses that are reviewed briefly together with a brief discussion about the origin of this rare entity.
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PMID:Cellular neurothekeoma with a plexiform morphology: a case report with a discussion of the plexiform lesions of the skin. 1730 11

Making a differential diagnosis to distinguish dermatofibrosarcoma protuberans (DFSP) from dermatofibroma (DF) is occasionally difficult. In those instances, CD34 and factor XIIIa have been used as valuable differential markers. The histogenesis of DFSP, however, remains uncertain and controversial, although it is generally thought to be a neuromesenchymal neoplasm. Nestin is an intermediate filament protein that was first observed in neuroectodermal stem cells. We investigated the expression of nestin in order to distinguish between DFSP and DF in combination with the use of conventional markers, CD34 and factor XIIIa. The nestin expression was investigated in tissue specimens from 16 DFSP cases and 30 DF cases. The expression of other differential markers such as CD34, factor XIIIa, CD163 and CD10 was also observed in these samples. Fifteen (94%) of 16 cases of DFSP showed the expression of nestin, whereas only four (13%) of 30 cases of DF showed the expression of nestin. Most of the DFSP cases showed a diffuse positive reaction in more than half of the tumor cells. In contrast, DF cases with nestin expression showed a partial positive reaction. Nestin is considered to be a useful and additional marker in combination with CD34, factor XIIIa and CD163 in order to distinguish between DFSP and DF. The frequent expression of nestin in DFSP may therefore indicate that DFSP is derived from putative, multipotent neuromesenchymal cells.
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PMID:Expression of nestin in dermatofibrosarcoma protuberans in comparison to dermatofibroma. 1870 29

Benign fibrous histiocytoma is one of the most frequent benign neoplasms mainly composed of a mixture of fibroblastic and histiocytic cells, especially found in the skin (dermatofibroma), particularly in the limbs. The diagnosis of cutaneous benign fibrous histiocytoma is generally easy; however, rare variants may be difficult to identify, and the diagnosis only confirmed after exhaustive histopathological examination. Thus, deep subcutaneous dermatofibroma may be difficult to distinguish from dermatofibrosarcoma protuberans and dermatofibroma with monster giant cells from malignant fibrous histiocytoma and atypical fibroxanthoma. We report a case of a 38-year-old woman with a painless swelling on the abdominal wall, which was totally excised and histopathologically diagnosed as subcutaneous atypical fibrous histiocytoma. The lesion was deeply located within the subcutaneous tissue and consisted of interlacing fascicles of predominant histiocyte-like spindle cells intermingled with pleomorphic giant cells with bizarre large nuclei (bilobed and multilobed) and prominent eosinophilic nucleoli. Only 1 mitotic figure was found in the whole lesion. Prominent hyaline collagen bundles surrounded by tumor cells were observed, predominantly at the periphery of the lesion. Immunohistochemical study showed positivity only for vimentin and factor XIIIa, whereas pan-keratins, actin, desmin, CD34, CD10, and S-100 protein were negative. Recognition of dermatofibroma is important, allowing sequential excision and optimal results. Definitive diagnosis, although especially difficult in our case, is established by characteristic histological and immunohistochemical criteria. To the best of our knowledge, we report the first case of subcutaneous fibrous histiocytoma with monster cells.
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PMID:Subcutaneous atypical fibrous histiocytoma. 1954 31

We herein present the clinicopathological features of an atypical cellular unguioblastic fibroma, a rare onychomatrical tumor, in an adult woman of Southeast Asian origin. The tumor displayed both diffuse hypercellularity and atypia in the mesenchymal component and the presence of large multinucleated cells with a ring-/floret-like arrangement of irregular and hyperchromatic nuclei. The mesenchymal cells displayed a patchy weak positive immunereactivity for CD34 (including atypical and wreath-like multinucleated cells) and for CD10 but were negative for low molecular weight cytokeratins, epithelial membrane antigen, CD68, factor XIIIa, smooth muscle actin, desmin, and HMB-45. The proliferative index as measured immunohistochemically (Mib-1/Ki-67) was 5%. The extent of these atypical histological features has not previously been described. One-year follow-up has been uneventful.
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PMID:Atypical cellular unguioblastic fibroma-A rare case with more atypical histological features than previously reported. 2051 82

Cutaneous clear cell tumors are a heterogeneous group of cutaneous neoplasms, which may show a wide range of histogenesis. We report the clinicopathological features of an agminated clear cell tumor, arising in a 67-year-old man, otherwise asymptomatic, with distinct histopathological and immunohistochemical features, which did not fit into any existing diagnostic categories. The patient presented with several skin-colored papules at the lateral and posterior aspects of the neck, which on histopathological examination showed circumscribed lobular aggregates of clear cells within the dermis. The immunohistochemical marker panel performed showed diffuse expression of vimentin, NKI-C3, and CD64 while revealing marked negativity for factor XIIIa, CD10, CD13, CD14, CD34, CD68, CD163, lysozyme, HMB45, Renal Cell Carcinoma antigen, calponin, h-caldesmon, Anti-alpha smooth muscle actin antibody [1 A4], S100, and pancytokeratin, leading the authors to postulate a monocytic origin.
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PMID:Agminated Clear Cell Tumor: An Impostor of PEComa and Distinctive Dermal Clear Cell Mesenchymal Neoplasm. 2782 2

Pseudomyogenic hemangioendothelioma (PHE) is a rare indolent vascular tumor that typically has a multifocal presentation and involves multiple tissue planes. This report describes a 34-year-old man with multiple infiltrated brown papules and plaques on his left leg that had evolved for 6 months. The skin biopsy revealed a dermal and subcutaneous neoplasm composed of fascicles of spindle cells with atypia and epithelioid cells with prominent nucleoli and abundant eosinophilic cytoplasm. There was no evidence of necrosis, and the mitotic rate was low. There was strong reactivity with cytokeratin AE1/AE3, ERG, and FLI1, multifocal reactivity with smooth muscle actin, and focal reactivity with CD31. There was no expression of keratin MNF116, CAM5.2, CD34, CAMTA1, S100-protein, epithelial membrane antigen, melan-A, HMB-45, factor XIIIa, HHV8, or CD10. The nuclei of neoplastic cells showed intact expression of INI1. The clinical, histological, and immunophenotypical aspects were consistent with a diagnosis of PHE. A lower limb CT scan showed lesions in the skin, muscle, and bone planes. The patient was sent to an oncology center, where he maintains regular clinical and imagiological follow-up.
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PMID:Pseudomyogenic hemangioendothelioma: a little-known tumor. 3056 38


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