Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts: Gene/Protein Disease Symptom Drug Enzyme Compound   Target Concepts: Gene/Protein Disease Symptom Drug Enzyme Compound

---

Query: EC:2.7.10.1 (ERK)
95,504 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The human homologue of the SEA oncogene has been mapped recently to chromosome band 11q13. While studying the possible involvement of this gene in the variant translocation t(9;22;11) (q34;q11;q13) in a case of chronic myelogenous leukemia, we identified novel polymorphisms for XbaI and SacI restriction enzyme sites in the SEA gene. Frequency of the polymorphic alleles was studied in 100 samples from healthy controls, 94 samples from patients with non-Hodgkin's lymphoma, 25 samples from patients with benign lymphadenopathy, and 38 samples from patients with chronic myelogenous leukemia. XbaI digestion showed a three-allele polymorphism with two frequent alleles A (8.0 kb) and B (9.2 kb) and a rare allele (5.8 kb). After SacI digestion the probe identified two primary genotypes. Genotype I showed two hybridizable DNA fragments, one each of 6.6 and 3.5 kb. In genotype II the 3.5 kb fragment was absent, instead two smaller fragments, one each of 1.9 kb and 1.6 kb were present. The 6.6 kb fragment (allele AA) had three polymorphic sites generating 6.2 kb fragment (allele BB), 7.4 kb fragment (allele CC), and 7.8 kb fragment (allele DD). Frequencies of the two genotypes and the four alleles followed Mendelian proportions in all the samples studied. Furthermore, this study shows the importance of restriction map analysis of DNA in the vicinity of the probe of an oncogene to distinguish natural polymorphisms from the disease-related rearrangements in the gene.
...
PMID:Novel restriction fragment length polymorphisms in the cellular oncogene SEA. 135 80

One-hundred-ninety-six patients with Stage III and IV Hodgkin's disease were prospectively randomized to receive either treatment with the methanol extraction residue of Bacillus Calmette-Guerin (MER/BCG) or no immunotherapy. Prior to the MER/BCG randomization, patients received six courses of induction and two years of maintenance chemotherapy so that a group with a presumptively low tumor burden could be established. Only patients achieving a complete remission were evaluated. During the first two years of immunotherapy, the MER/BCG group had a relapse frequency twice that of controls. The overall crude relapse frequency and disease-free survival were similar between the two treatment groups. The MER/BCG dose schedule used in this study was associated with a high frequency of unacceptable toxicity. Ulcerations of greater than 1 cm occurred in one-third of the patients with associated pain, fever, and occasional lymphadenopathy. A high degree of patient noncompliance (36%) was observed. Age (P = 0.002), prior radiotherapy (P = 0.032), and chemotherapy (P = 0.044) were prognostic factors found to significantly influence remission duration. These factors were balanced between patients treated with immunotherapy and those who were not. MER/BCG therapy did not significantly delay or prevent relapse.
...
PMID:Effect of methanol extraction residue of Bacillus Calmette-Guerin in advanced Hodgkin's disease. 680 82

Non-Hodgkin's lymphoma (NHL) occasionally involves the placenta, and information of such occurrence should be useful for management of the mother and fetus. We report the first case of anaplastic large cell lymphoma (ALCL) disseminated to the placenta. The diagnosis was made via excisional biopsy of cervical lymphadenopathy in a 20-year-old woman at 27 weeks' gestation. Involvement of the placenta was noted on gross examination after cesarean section delivery of a girl at 30 weeks' gestation. The ALCL was microscopically confined to intervillous spaces in a manner similar to previous reports of other NHLs. The immunophenotype was characteristic (CD30+, EMA+, BNH9+), and the now frequently associated t(2;5)(p23;q35) translocation with this lymphoma was detected by the recently produced monoclonal antibody ALK1 against the nucleophosmin/anaplastic lymphoma kinase (NPM/ALK) chimeric protein. Complete remission was induced in the mother after delivery. Both mother and child are healthy at 10 years' follow-up. The case is reported in light of the sparse literature on lymphomatous involvement of the placenta.
...
PMID:Anaplastic large cell lymphoma of maternal origin involving the placenta: case report and literature survey. 933 Dec 98

The subgroup of T/null-cell primary systemic anaplastic large cell lymphoma that expresses anaplastic lymphoma kinase (ALK) constitutes a distinctive clinicopathologic entity that exhibits a broad morphologic spectrum. The examples predominated by small cells or showing a mixed cell population can be difficult to recognize as being neoplastic. We report four such cases with a remarkably hypocellular granulation tissue-like appearance, mimicking an inflammatory or reparative process. All patients were young and presented with lymphadenopathy in multiple sites. The lymph node biopsies showed a hypocellular appearance, with wide separation of the small to medium-sized lymphoid cells by edematous or fibromyxoid stroma. There were interspersed spindly neoplastic cells resembling myofibroblasts, sometimes forming short, sweeping fascicles, as well as histiocytes. Occasional large cells with atypical nuclei were identified. The larger lymphoid cells tended to form cuffs around the venules. In two cases, the capsule and fibrous trabeculae were markedly broadened with increased spindly cells, mimicking inflammatory pseudotumor of lymph node. Immunostaining showed dispersed and clustered CD30+ ALK+ cells, confirming a diagnosis of anaplastic large cell lymphoma. In conclusion, a diagnosis of hypocellular anaplastic large cell lymphoma requires a high index of suspicion. The young age of the patients and the presence of perivascular cuffs of larger lymphoid cells should provide the strongest clues to the correct diagnosis.
...
PMID:Hypocellular anaplastic large cell lymphoma mimicking inflammatory lesions of lymph nodes. 1107 56

Inflammatory pseudotumors (IPTs) of the lymph node and spleen are an uncommon, benign cause of lymphadenopathy and/or splenomegaly that often bear striking clinicopathologic similarities to the inflammatory myofibroblastic tumors (IMTs) found in soft tissues. These tumors have classically been grouped together under the umbrella category of "inflammatory pseudotumor." Recent evidence shows that IMTs are in fact neoplastic processes that often harbor balanced chromosomal translocations involving the ALK kinase gene. These translocations result in expression of ALK kinase in IMTs as assessed by immunohistochemical studies. However, the relationship between IMT and IPT of the lymph node and spleen is uncertain. To determine if ALK tyrosine kinase expression is also present in IPT, 13 cases of IPT (9 involving lymph nodes, 4 splenic lesions) were examined for the presence of ALK tyrosine kinase by immunohistochemical staining on paraffin-embedded tissue. In addition, in situ hybridization studies for Epstein-Barr virus--encoded RNAs (EBER) and immunoperoxidase studies for human herpesvirus-8 (HHV8)--specific proteins were performed. All cases had clinical, morphologic, and immunophenotypic findings typical of IPT and had varying proportions of fibroblastic and inflammatory components. Age ranged from 11 to 75 (median, 40) years; 8 subjects were male, and 5 were female. None of the cases (0 of 13) had positive staining for ALK kinase or HHV8, and in 1 a lymph node (1 of 13) was focally positive for EBV (EBER) by in situ hybridization. The absence of ALK kinase as detected by immunohistochemical studies in IPT of the lymph node and spleen suggests that this entity is biologically distinct from the histologically similar IMT.
...
PMID:Inflammatory pseudotumor of lymph node and spleen: an entity biologically distinct from inflammatory myofibroblastic tumor. 1177 73

A t(2;5) (p23;q35) chromosomal translocation can be found in a high percentage of anaplastic large-cell lymphomas (ALCL). This genetic abnormality leads to the expression of the NPM-ALK fusion protein, which encodes a constitutively active tyrosine kinase that plays a causative role in lymphomagenesis. Employing a modified infection/transplantation protocol utilizing an MSCV-based vector, we were able to reproducibly induce two phenotypically different lymphoma-like diseases dependent on the retroviral titers used. The first phenotype presented as a polyclonal histiocytic malignancy of myeloid/macrophage origin with a short latency period of 3-4 weeks. Clinically, the diseased mice showed rapidly progressive wasting, lymphadenopathy and pancytopenia. Mice displaying the second phenotype developed monoclonal B-lymphoid tumors with a longer latency of approximately 12-16 weeks, primarily involving the spleen and the bone marrow, with less extensive lymph node but also histologically evident extranodal organ infiltration by large immature plasmoblastic cells. The described retroviral mouse model will be useful to analyse the role of NPM-ALK in lymphomagenesis in vivo and may contribute to the development of new treatment options for NPM-ALK induced malignancies.
...
PMID:The oncogenic fusion protein nucleophosmin-anaplastic lymphoma kinase (NPM-ALK) induces two distinct malignant phenotypes in a murine retroviral transplantation model. 1287 8

TNF receptor (TNFR) superfamily members, CD40, and BAFFR play critical roles in B cell survival and differentiation. Genetic deficiency in a novel adaptor molecule, Act1, for CD40 and BAFF results in a dramatic increase in peripheral B cells, which culminates in lymphadenopathy and splenomegaly, hypergammaglobulinemia, and autoantibodies. While the B cell-specific Act1 knockout mice displayed a similar phenotype with less severity, the pathology of the Act1-deficient mice was mostly blocked in CD40-Act1 and BAFF-Act1 double knockout mice. CD40- and BAFF-mediated survival is significantly increased in Act1-deficent B cells, with stronger IkappaB phosphorylation, processing of NF-kappaB2 (p100/p52), and activation of JNK, ERK, and p38 pathways, indicating that Act1 negatively regulates CD40- and BAFF-mediated signaling events. These findings demonstrate that Act1 plays an important role in the homeostasis of B cells by attenuating CD40 and BAFFR signaling.
...
PMID:Act1, a negative regulator in CD40- and BAFF-mediated B cell survival. 1548 34

Necropsy on a 9-month-old ARK/J mouse, presenting with a large thymic mass as well as generalized hypertrophic lymphadenopathy, revealed a generalized thymic lymphoma on histopathological examination. This strain of mouse is genetically predisposed to developing this type of neoplasm.
...
PMID:[Generalized thymic lymphoma in a 9-month-old ARK/J mouse]. 1551 Jun 88

An 11 year-old girl presented with two large abdominal masses in the left flank and epigastrium and left supraclavicular lymphadenopathy. Subsequent investigations led to the diagnosis of metastatic perivascular epithelioid cell tumor (PEComa) arising from the left kidney. Effective treatment for this rare tumor is not yet known. The tumor did not respond to an initial treatment of two cycles of a dacarbazine (DTIC) based regimen. She was placed on a trial of imatinib mesylate based on tumor expression of c-KIT, a tyrosine kinase targeted by this drug. This report highlights the first documented case of the use of imatinib for PEComa. Lack of response and adverse effects of the drug required discontinuation of therapy.
...
PMID:Lack of response of a metastatic renal perivascular epithelial cell tumor (PEComa) to successive courses of DTIC based-therapy and imatinib mesylate. 1570 92

A 33-years-old man was diagnosed as having undifferentiated carcinoma presenting with right neck lymphadenopathy in December 2000. He obtained complete remission (CR) following chemotherapy, radiation and lymphadenectomy on the right neck. He had multiple para-aorta lymphadenopathy and splenomegaly in December 2001. An open-abdominal lymph node biopsy was performed from which a diagnosis of anaplastic large cell lymphoma was made. CR was achieved with biweekly CHOP, however, the patient suffered from a relapse twice. He underwent allogeneic peripheral blood stem cell transplantation (PBSCT) from his HLA-matched sister while in non-CR in November 2002. Engraftment was achieved on day 14, and at the same time, complete chimerism was confirmed. Acute grade III graft-versus-host disease (GVHD) developed and was controlled with cyclosporine A and prednisolone. Extensive chronic GVHD was subsequently observed and required systemic immunosuppression. His condition returned to CR after the PBSCT and he sustained complete chimerism. He suddenly died of fulminant thrombotic microangiopathy seven months after the PBSCT. The tumor cells were ALK-positive, CD30-negative and JH rearrangement was detected, and were therefore classified as diffuse large B-cell lymphoma with expression of ALK according to the WHO classification, though they differed from this subtype in some points. Although this case was refractory for chemotherapy with a complex karyotype, the graft-versus-lymphoma effect might have contributed to the sustained CR following the PBSCT.
...
PMID:[CD30-negative diffuse large B-cell lymphoma expressing ALK]. 1644 Jul 42


1 2 3 4 5 6 7 8 9 10 Next >>

---