Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UNIPROT:P10415 (Bcl-2)
 33,771 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Generalized basaloid follicular hamartoma syndrome (GBFHS) is a rare, recently-described, autosomal-dominantly inherited disorder that presents with disseminated milia, palmoplantar pitting, hypotrichosis and basaloid follicular hamartomas (BFH). BFH is a benign adnexal tumor that resembles basal cell carcinoma (BCC). In this study, we report two cases of GBFHS and stain BFH, a vellus hair hamartoma (VHH) and a neurofollicular hamartoma (NH) with CD34, bcl-2 and CD10 to characterize and compare the staining patterns of these follicular tumors. Standard immunohistochemistry labeling with CD34, bcl-2 and CD10 was performed on paraffin-embedded, formalin-fixed tissue sections of five BFH (four for CD10), one VHH and one NH. CD34 stromal staining was observed in all specimens. Bcl-2 stained the outermost cell layers of the basaloid nests in all specimens. CD10 stained the peritumoral stroma of all specimens. The BFH, NFH and the VHH showed CD10 staining of matrical cells. CD34 and CD10 stain peritumoral stroma of BFH, VHH and NH. Bcl-2 stains the outermost cell layer of these tumors. CD10 was also observed to stain matrical cells. These results show the similarities in differentiation between these benign follicular neoplasms and trichoepithelioma.
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PMID:Bcl-2, CD34 and CD10 expression in basaloid follicular hamartoma, vellus hair hamartoma and neurofollicular hamartoma demonstrate full follicular differentiation. 1947 35

Keeping in view the fact that a single acquired genetic abnormality "Bcr-Abl chimeric gene" accompanied by elevated telomerase activity has been widely recognized to be responsible for the leukemic myelopoiesis observed in chronic myeloid leukemia (CML), the present study was addressed to understand as to how selective and specific knock-down of human telomerase reverse transcriptase (hTERT) gene within mononuclear cells derived from untreated CML subjects could influence the apoptotic, genotypic (such as Bcr-Abl; C-myc; Bcl-2; IL-6; GMCSF; IL-3; and acetylated H(3) and H(4)), and phenotypic (such as CD34 and CD89) characteristics of these cells. Based upon these results, we propose that hTERT gene-based drug design may be useful in the treatment of leukemic myelopoiesis.
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PMID:Functional genomics of hTERT gene in leukemic myelopoiesis. 1840 31

Inflammatory myofibroblastic tumor is a rare lesion composed of myofibroblastic spindle cells accompanied by inflammatory infiltrate. The objective of this paper is to report an uncommon case of inflammatory myofibroblastic tumor located in the alveolar mucosa of the mandible. A 33-year-old male presented an asymptomatic tumoral lesion, firm, pedunculated, pink-colored, covered by smooth mucosa, with focal ulceration, measuring 30x20x20 mm, located in the left posterior alveolar mucosa. Clinical diagnosis was soft tissue tumor. An excisional biopsy was made. Microscopic examination showed compact fascicular spindle cells proliferation with a diffuse inflammatory infiltrate of plasma cells, lymphocytes, and eosinophils. Large ganglion-like cells were observed. The lesional cells were immunopos-itive to vimentin, a-smooth muscle actin, muscle specific actin, and CD68. Negative immunostain was observed to S-100, Bcl-2, Ki-67, desmin, CD34, and cytokeratin. A diagnosis of inflammatory myofibroblastic tumor was performed. After 28 months of follow-up there was no recurrence. Although no evidence of oral inflammatory myofibroblastic tumor recurrence or malignant transformation has been reported, it has been observed that in inflammatory myofibroblastic tumor of other regions a prolonged follow-up is necessary after surgical excision.
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PMID:Inflammatory myofibroblastic tumor of the alveolar mucosa of the mandible. 1842 72

Spontaneous apoptosis of bone marrow erythroid precursors accounts for the anemia that characterizes most low-grade myelodysplastic syndromes (MDS). We have shown that death of these precursors involved the Fas-dependent activation of caspase-8. To explore the pathway leading from caspase-8 activation to apoptosis, we transduced MDS bone marrow CD34(+) cells with a lentivirus encoding wild-type (WT) or endoplasmic reticulum (ER)-targeted Bcl-2 protein before inducing their erythroid differentiation. Both WT-Bcl-2 and ER-targeted Bcl-2 prevented spontaneous and Fas-dependent apoptosis in MDS erythroid precursors. ER-targeted Bcl-2 inhibited mitochondrial membrane depolarization and cytochrome c release in MDS erythroid precursors undergoing apoptosis, indicating a role for the ER in the death pathway, upstream of the mitochondria. MDS erythroid precursors demonstrated elevated ER Ca(2+) stores and these stores remained unaffected by ER-targeted Bcl-2. The ER-associated protein Bcl-2-associated protein (BAP) 31 was cleaved by caspase-8 in MDS erythroid precursors undergoing apoptosis. The protective effect of ER-targeted Bcl-2 toward spontaneous and Fas-induced apoptosis correlated with inhibition of BAP31 cleavage. A protective effect of erythropoietin against Fas-induced BAP31 cleavage and apoptosis was observed. We propose that apoptosis of MDS erythroid precursors involves the ER, downstream of Fas and upstream of the mitochondria, through the cleavage of the ER-associated BAP31 protein.
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PMID:Spontaneous and Fas-induced apoptosis of low-grade MDS erythroid precursors involves the endoplasmic reticulum. 1861 9

Interleukin-24 (IL-24) has been shown to be a tumor-suppressor gene and the protein product found to be constitutively expressed by melanocytes, nerve cells, and some primary melanomas. The potential effect of adenovirus (AdV)-mediated IL-24 gene therapy was explored on human pancreatic carcinoma by using a pancreatic carcinoma cell line, patu8988. A recombinant adenovirus, AdVGFP/IL-24, expressing the marker, green fluorescent protein (GFP), and the tumor-suppressor gene, IL-24, was constructed. AdVGFP/IL-24 treatment of pancreatic carcinoma cells in vitro significantly induced pancreatic carcinoma cell cytotoxicity and apoptosis, compared with AdVGFP without IL-24 expression. In nude mice bearing patu8988 tumors, intratumoral injections of AdVGFP/IL-24 significantly inhibited pancreatic carcinoma growth. In addition, the molecular mechanism of tumor suppression was elucidated by downregulating the expression of vascular endothelial growth factor, CD34, and Bcl-2, as well as inhibiting tumor angiogenesis. Therefore, AdVGFP/IL-24 has the potential to serve as a novel tool for pancreatic carcinoma gene therapy.
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PMID:Inhibition of pancreatic carcinoma growth by adenovirus-mediated human interleukin-24 expression in animal model. 1877 46

To elucidate whether caspase activation is involved in megakaryopoiesis, we characterized megakaryocytes (MKs) in vav-bcl-2 transgenic (Tg) mice, in which Bcl-2 is overexpressed in hematopoietic cells. To exclude the effect of splenomegaly in Tg mice on megakaryopoiesis, splenectomy was performed. After splenectomy, basal platelet counts in peripheral blood were not significantly different between Tg and wild-type (WT) mice. However, when experimental thrombocytopenia was induced by injecting 5-fluorouracil into splenectomized mice, overshoot of platelet counts during the recovery phase was hardly observed in Tg mice. Analyses of MK ploidy during the recovery phase showed that MKs less than 16 N ploidy were significantly decreased in Tg mice, suggesting that MK supply from progenitors is impaired. Supporting this, differentiation of CD34-/c-kit+/Sca-1+/Lineage- stem cells into MKs was significantly hampered in Tg mice, whereas megakaryocyte-erythroid progenitors (MEPs) normally differentiated into MKs. It suggests that differentiation into MKs is impaired in Tg mice before the stage of MEP. Furthermore, MK colony formation in WT cells was dose-dependently inhibited in the presence of a caspase inhibitor. Contrary, Bcl-2-overexpressing MKs showed normal ability for in vitro platelet production. We thus believe that caspase activation is involved in the differentiation of progenitors into megakaryocytic lineage but not in platelet production.
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PMID:Caspase activation is involved in early megakaryocyte differentiation but not in platelet production from megakaryocytes. 1921 31

As a result of the progressive decrease in efficacy of drugs used to treat Parkinson's disease (PD) and the rapid development of motor complications, effective alternative treatments for PD are required. In a 6-hydroxydopamine (6-OHDA)-induced Parkinson's rat model, intracerebral peripheral blood stem cell (CD34(+)) (PBSC) transplantation significantly protected dopaminergic neurons from 6-OHDA-induced neurotoxicity, enhanced neural repair of tyrosine hydroxylase neurons through up-regulation of Bcl-2, facilitated stem cell plasticity, and attenuated activation of microglia, in comparison with vehicle-control rats. The 6-OHDA-lesioned hemi-Parkinsonian rats receiving intrastriatal transplantation of PBSCs also showed: 1) enhanced glucose metabolism in the lesioned striatum and thalamus, demonstrated by [(18)F]fluoro-2-deoxyglucose positron emission tomography (FDG-PET), 2) improved neurochemical activity as shown by proton magnetic resonance spectroscopy ((1)H-MRS), and 3) significantly reduced rotational behavior in comparison with control lesioned rats. These observations might be explained by an up-regulation of growth-associated protein 43 (GAP-43) expression because improvements in neurological dysfunction were blocked by injection of MK-801 in the PBSC-treated group. In addition, a significant increase in neurotrophic factor expression was found in the ipsilateral hemisphere of the PBSC-treated group. In summary, this protocol may be a useful strategy for the treatment of clinical PD.
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PMID:Induction of GAP-43 modulates neuroplasticity in PBSC (CD34+) implanted-Parkinson's model. 1923 91

Malignant solitary fibrous tumor (MSFT) is a rare neoplasm. Three cases of MSFT with unusual features, including 1 pleural and 2 extrapleural, are reported. A 64-year-old woman with a large right thoracic MSFT and episodes of severe hypoglycemia experienced resolution of her hypoglycemia immediately after resection of the MSFT. A 27-year-old woman with primary retroperitoneal MSFT had pulmonary metastases 10 months after resection of the primary tumor. A 54-year-old man with an intracranial solitary fibrous tumor suffered from multiple pulmonary metastases and local recurrence 21 and 28 months after resection of the primary tumor, respectively. All 3 cases of solitary fibrous tumor displayed malignant features. The tumor cells in each case were positive for CD34 and Bcl-2, but negative for cytokeratin, smooth muscle actin, S-100, and c-kit. In addition, the tumor cells in the case with concomitant hypoglycemia were strongly positive for insulin-like growth factor-II. The histopathologic diagnostic criteria for MSFT, the differential diagnosis with other spindle cell tumors, and the mechanism of MSFT-derived hypoglycemia via insulin-like growth factor-II are discussed.
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PMID:Malignant solitary fibrous tumor: report of 3 cases with unusual features. 1934 55

A 58-year-old woman without any clinical symptoms had a routine health check-up, during which a mass in the head of the pancreas was detected by US. Abdominal US showed a round, hypoechogenic nodule of the pancreatic head. The tumor measured 3cm in diameter and contained an anechoic area. CT revealed a well-demarcated tumor in the pancreatic head. The tumor was partially enhanced by contrast medium, but the left portion of the tumor, which US had shown to be an anechoic area was not enhanced. Imaging findings suggested an endocrine tumor with cystic changes. A pylorus-preserving pancreatoduodenectomy was performed. The tumor was composed of spindle-shaped cells. There was a typical patternless growth of short fascicles and a short storiform arrangement of cells in the stroma with varying degrees of collagenization. Immunohistochemically, the tumor cells were positive for CD34 and Bcl-2. Based on the light microscopic morphology and immunostaining profile, the tumor was classified as a solitary fibrous tumor (SFT) of the pancreas.
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PMID:[A case of solitary fibrous tumor of the pancreas]. 1957 17

We describe clinical, morphologic, and immunohistochemical features of 21 cases of solitary fibrous tumor presenting in the oral cavity. There were 9 male and 12 female patients with a median age of 51 years (range 37-83). The most common locations included the buccal mucosa (the most common site), lip, maxillary or mandibular vestibule and tongue. Histopathologic examination showed well-circumscribed tumors with two well-defined patterns: the classic pattern with densely cellular areas alternating with hypocellular areas in a variably collagenous, vascular stroma and a more uniformly sclerotic pattern with only subtle classic areas. The spindle-shaped neoplastic cells consistently showed immunoreactivity for antibodies directed against CD34. Five of nineteen cases (26%) were reactive for CD99 and 19 of 19 for Bcl-2. Follow-up information was available in 17 cases and averaged 54 months, with no evidence of recurrence or metastasis in any of these patients. Awareness that solitary fibrous tumor may present in the oral cavity is important so that confusion with other spindle cell neoplasms can be avoided. We also briefly describe the differential diagnosis and compare this series, the largest single series of intraoral SFT, to cases previously reported in the literature.
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PMID:Solitary fibrous tumor of the oral cavity: clinicopathologic and immunohistochemical study of 21 cases. 1964 41


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