Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0155339 (Brown)
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We report the case of a woman suffering from progressive bulbopontine paralysis in whose the first symptom, bilateral hypoacousia, began in childhood. This clinical picture is that of the Brown-Vialetto-Van Laere (BVVL) syndrome. Anti-ganglioside GM1 antibodies were moderately elevated in this patient. Intravenous immunoglobulins produced little benefit. The main clinical characteristics of 29 BVVL patients reported in literature are reviewed, and the pathological significance of anti-GM1 antibodies is discussed in the context of this disorder.
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PMID:[Brown-Vialetto-Van Laere syndrome: a case with anti-ganglioside GM1 antibodies and literature review]. 977 26

The transplanted limb contains bone marrow tissue. The hematopoietic cells contained in the bone of the graft normally differentiate after transplantation and can be released to the recipient. The cells migrate to the recipient bone marrow cavities and lymphoid organs. This causes the immune reaction between the donor and the recipient, which develops not only in the graft itself but also in the recipient immune organs where donor bone marrow cells home. The purpose of this study was to investigate the process of migration of the hematopoietic cells from the donor limb to the recipient bone marrow cavities and lymphoid tissues. The questions the authors asked were: what is the rate of release of bone marrow cells from the transplanted bone, where do the released bone marrow cells home in the recipient, how fast are donor bone marrow cells rejected by the recipient, and can some bone marrow cells homing in the recipient tissues survive and create a state of microchimerism. Experiments were performed on Brown Norway and Lewis inbred rat strains (n = 30). Limb donors received intravenous chromium-51-labeled bone marrow cells. Twenty-four hours later, the limb with homing labeled bone marrow cells was transplanted to an allogeneic or syngeneic recipient. The rate of radioactivity of bone marrow cells released from the graft and homing in recipient tissues was measured after another 24 hours. To eliminate factors adversely affecting homing such as the "crowding effect" and allogeneic elimination of bone marrow cells by natural killer cells, total body irradiation and antiasialo-GM1 antiserum were applied to recipients before limb transplantation. In rats surviving with the limb grafts for 7 and 30 days, homing of donor bone marrow cells was studied by specific labeling of donor cells and flow cytometry as well as by detecting donor male Y chromosome. The authors found that transplantation of the limb with bone marrow in its natural spatial relationship with stromal cells and blood perfusion brings about immediate but low-rate release of bone marrow cells and their migration to recipient bone marrow and lymphoid tissues. Large portions of allogeneic bone marrow cells are rapidly destroyed in the mechanism of allogeneic elimination by radioresistant but antiasialo-GM1-sensitive natural killer cells. Some transplanted bone marrow cells remain in the recipient's tissues and create a state of cellular and DNA microchimerism. A low number of physiologically released donor bone marrow cells do not seem to adversely affect the clinical outcome of limb grafting. Quite the opposite, a slight prolongation of the graft survival time was observed.
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PMID:Biological aspects of limb transplantation: I. Migration of transplanted bone marrow cells into recipient. 1457 94

Soil enzymes influence the distribution of organic matter (OM) and play a decisive role in the retention of OM in soil ecosystems. The present study was conducted to assess the role of oxidative and hydrolytic enzymes in OM decomposition under dry and submerged moisture conditions and to examine factors affecting their activity in Red and Brown soils. An incubation experiment of 6 mo duration was performed in a randomized complete block design with 10 treatments in triplicates. Rice straw (RS) and green manure (GM) were used at three rates (0, 5, and 25 mg g soil, expressed as control [CK], RS1, RS2, GM1, and GM2, respectively). The soils were maintained at two water levels: 25% (W1) and 200% (W2). All soil chemical properties and enzymatic activities were measured by standard methods. We found that the activities of soil phenol oxidase and catalase in the Brown soil were 2- and 1.5-fold higher than in the Red soil, respectively. This led to high OM decomposition, resulting in 1.23- and 1.2-fold higher carbon (C) and nitrogen (N) mineralization and 1.23- and 1.21-fold more dissolved organic carbon (DOC) and dissolved organic nitrogen (DON) in the Brown soil. In contrast, low oxidative enzyme activities in the Red soil decreased C and N mineralization, DOC, DON, reducing sugars (2.58-fold), and amino acids (2.22-fold) but increased the phenolic compounds (1.36-fold). The hydrolytic enzymes (i.e., urease and neutral phosphatase) showed significant correlation ( < 0.01) to amino acids and reducing sugars but showed no correlation to phenolic compounds and soil organic matter (SOM). Conversely, oxidative enzymes (i.e., phenol oxidase and catalase) showed significant correlation ( < 0.01) to phenolic compounds, amino acids, reducing sugars, and SOM. Our results suggest that oxidative enzymes are key components of the pathways involved in the breakdown of organic compounds and OM in soils and consequently act as an "enzymatic latch." We observed that soil water and pH have significant ( < 0.01) effects on soil enzyme activity.
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PMID:Oxidative enzymes, the ultimate regulator: implications for factors affecting their efficiency. 2560 18