Rabbit Anti-Human FHL2 Polyclonal Antibody

Background. Post-transplant outcomes of hemophagocytic lymphohistiocytosis (HLH) patients were analyzed in Japan where Epstein-Barr virus (EBV)-associated severe forms are problematic. Methods. Fifty-seven patients (43 familial HLH [12 FHL2, 11 FHL3, 20 undefined], 14 EBV-HLH) who Underwent Stem Cell transplantation (SCT) between 1995 and 2005 were enrolled based oil the nationwide registration. Results. Fifty-seven patients underwent 61 SCTs, including 4 consecutive SCTs. SCTs were employed using allogeneic donors in 93% of cases (allo 53, twin 1, auto 3). Unrelated donor cord blood transplantation (UCBT) was employed in half of cases (21 FHL, 7 EBV-HLH). Reduced intensity conditioning was used in 26% of cases. The 10-year overall Survival rates (rnedian +/- SE%) were 65.0 +/- 7.9%. in FHL and 85.7 +/- 9.4%. in EBV-HLH patients, respectively. The Survival of UCBT recipients was >65%. in both FHL and EBV-HLH patients. Three Out Of four patients were alive with Successful engraftment after second UCBT. FHL patients showed a Poorer Outcome due to early treatment-related deaths (<100 days, seven patients) and a higher incidence of sequelae than EBV-HLH patients (P=0.02). The risk of death for FHL patients having received all unrelated donor bone marrow transplant was marginally higher than that for a related donor SCT (P=0.05) and that for UCBT (P=0.07). Conclusions. EBV-HLH patients had a better prognosis after SCT than FHL patients. FHL patients showed either an equal or better Outcome even after UCBT compared with the recent reports. UCB might therefore be acceptable as all alternate SCT source for HLH patients, although the optimal conditioning remains to be determined. Pediatr Blood Cancer 2010;54:299-306. (C) 2009 Wiley-Liss, Inc.

LIM domain containing proteins play critical roles in animal development and cellular differentiation. Here, we describe the cloning and expression patterns of three members of the four and a half LIM domain-only protein family, FHL1, 2, and 3, from mouse. A comparison of embryonic expression patterns of these three highly-related genes indicates that they are expressed in an overlapping pattern in the developing cardiovascular system, and skeletal muscle. In adult tissues, the three genes are expressed in a predominant and overlapping manner in cardiac and skeletal muscle. Of the three genes, FHL2 appears to have the most restricted expression pattern during development, in heart, blood vessels, and skeletal muscle. Expression in heart is highest in cardiac septa and in the region adjacent to the atrio-ventricular ring, suggesting a potential role in septation or conduction system development. In the heart, FHL1 expression was observed strongly in developing outflow tract, and to a lesser extent in myocardium. FHL3 displays low and ubiquitous expression during mouse development. Cardiac ventricular expression of FHL1, but not FHL2 or FHL3, was upregulated in two mouse models of cardiac hypertrophic and dilated cardiomyopathy. Taken together, these data indicate the potential importance of this FHL family in the development and maintenance of the cardiovascular system and striated muscle, and suggest that FHL1 may play a role in the development of heart disease. (C) 2000 Elsevier Science Ireland Ltd. All rights reserved.