Findings from University of Siena, Medical Department provide new insights into Krabbe disease
2007 NOV 12 -- "Background Inflammatory and/or immune activation occurs both in animal models (twitcher mice) and in the brain of patients with Globoid cell leukodystrophy (GLD) or Krabbe's disease (KD). In this study we evaluated in vitro the cytokine profile of KD patients and the effect of psychosine, the toxic metabolite which plays a role in the demyelination process in these patients," scientists in Siena, Italy report. "Materials and methods We studied cytokine production by peripheral blood mononuclear cells (PBMCs) isolated from four KD patients, diagnosed on the basis of clinical criteria. Cells were cultured and stimulated with appropriate agents and the supernatants collected before and after the addition of psychosine. Tumour necrosis factor-alpha (TNF-alpha), interleukin-8 (IL-8) and monocyte chemoattractant factor (MCP)-1) production was evaluated (ELISA method) and compared with a group of 10 normal subjects. Results We found a significant increase of TNF-alpha release by PBMCs of KD patients compared with healthy subjects; TNF-alpha production was significantly increased after LPS addition. Psychosine was able to induce a further significant increase (P < 0.05) only in cells obtained from KD patients and not from control subjects. No changes were found in IL-8 and MCP-1 production," wrote A.L. Pasqui and colleagues, University of Siena, Medical Department. The researchers concluded: "Conclusions The increased TNF-alpha production permits us to confirm the presence of an inflammatory-immune stimulus in KD patients, which may be induced and potentiated by the pathogenetic metabolite psychosine." Pasqui and colleagues published their study in European Journal of Clinical Investigation (Increased TNF-alpha production by peripheral blood mononuclear cells in patients with Krabbe's disease: effect of psychosine. European Journal of Clinical Investigation, 2007;37(9):742-745). For more information, contact A.L. Pasqui, University of Siena, Sect Internal Medical, Dept. of Internal Medicine & Immunology, Via Laterina 8, I-53100 Siena, Italy. Publisher contact information for the European Journal of Clinical Investigation is: Blackwell Publishing, 9600 Garsington Rd., Oxford OX4 2DQ, Oxon, England. Keywords: Italy, Siena, Globoid Cell Leukodystrophy, Krabbe Disease, University of Siena, Medical Department. This article was prepared by Pain & Central Nervous System Week editors from staff and other reports. Copyright 2007, Pain & Central Nervous System Week via NewsRx.com.
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