Cholesteryl ester storage disease (CESD) and Wolman disease (WD) are both autosomal recessive disorders associated with reduced activity and genetic defects of lysosomal acid lipase (LAL). The strikingly more severe course of WD is caused by genetic defects of LAL that leave no residual enzymatic activity. Mutations at the exon 8/intron 8 transition of the LAL gene have been identified in sever...

Deficiency of lysosomal acid lipase (LAL) leads to either Wolman disease (WD) or the more benign cholesteryl ester storage disease (CESD). To identify the molecular basis of the different phenotypes we have characterised the LAL gene mutations in three new patients with LAL deficiency. A patient with WD was homozygote for a null allele Y303X. The other two patients, with CESD, presented either ...

Male wild house mice, selected for short (SAL) and long (LAL) attack latency, show distinctly different behavioral strategies in coping with environmental challenges. In this study, we tested the hypothesis that this difference in coping style is associated with a differential stress responsiveness of the hypothalamic-pituitary-adrenal (HPA) system. SAL rather than LAL mice showed a clear fluct...

Mechanical load regulates ventricular growth, function, and structure from the earliest stages of cardiac morphogenesis through senescence. Dramatic changes in cardiac form and function have been defined for developing cardiovascular systems, and changes in mechanical loading conditions can produce structural malformations such as left heart hypoplasia. To date, relatively little is known regar...

They can transform the nature of education – where and how learning takes place and the roles of students and teachers in the learning process. The professional development of teacher educators in the area of ICT integration is essential. Unless teacher educators model effective use of technology in their own classes, it will not be possible to prepare a new generation of teachers who effective...

The functional roles of neutral lipids are poorly understood in the lung. Blocking cholesteryl ester and triglyceride metabolism in lysosomal acid lipase gene knockout mice (lal-/-) resulted in a high level of neutrophil influx in the lungs as early as 2 mo of age. Bronchoalveolar macrophages appeared foamy and gradually increased in number with age progression. Affymetrix GeneChip array analys...

Lysosomal Acid Lipase Regulates Myeloid-derived Suppressor Cells to Control Cancer Cell Proliferation and Metastasis. Inflammation critically contributes to cancer growth and metastasis, in which myeloid-derived suppressor cells (MDSCs) are an important participant. MDSCs are known to suppress immune surveillance to promote tumorigenesis [1]. Lysosomal acid lipase (LAL), a critical enzyme in co...

Abstract The demand is growing for new nanoscience-based technologies with unique properties that are different from traditional wet-chemical techniques. In recent years, laser ablation in liquid (LAL) has attracted increasing attention nanomaterial synthesis, which rapidly advanced both fundamental research and applications. Compared to other techniques, LAL easy set up simple perform. A large...

Synthesis of four and five coordinated aluminum amidinate (PhC(NtBu)2=L) compounds are reported herein along with their single-crystal X-ray structures detailed characterizations using both, experimental computational techniques. LAlCl2 (1) L2AlCl (2) were synthesized by treating AlCl3 PhC(NtBu)2Li in an equivalent ratio 1 : 2, respectively. [LAl(C2H5)Cl] (3) was prepared from a reaction AlCl2(...

Myeloid-derived suppressor cells (MDSCs) in mouse are inflammatory cells that play critical roles in promoting cancer growth and metastasis by directly stimulating cancer cell proliferation and suppressing immune surveillance. In order to facilitate characterization of biochemical and cellular mechanisms of MDSCs, it is urgent to establish an "MDSC-like" cell line. By cross breeding of immortom...