A question of concentration: The condensation of ethyleneurea and formaldehyde can be controlled perfectly by the HCl concentration to provide either a hemicucurbit[6]uril 1, which functions as a host, or hemicucurbit[12]uril 2, which acts as a gelating agent, in yields of 94 and 93 %, respectively.

Background: Aldosterone induces cardiac fibrosis in experimental animal models, but only limited information is available on the association between aldosterone and left ventricular (LV) hypertrophy in human beings. The aim of the present study was to determine the role of aldosterone in LV geometry and to investigate other types of target organ damage in hypertensive patients.

Pacific bluefin tuna larvae (Thunnus thynnus) were experimentally reared from 2-day-old yolk-sac larvae through 30-day-old early juveniles in June and July 1994. The larvae initially fed on rotifers on Day 3 and Artemia nauplii, fish eggs and larvae around Day 13, and thereafter were fed Artemia larvae and an artificial diet. The larvae had transformed to the juvenile stage after 30 days. The primitive digestive system differentiated on Day 3. The gastric gland and pyloric caeca first appeared on Day 11 and 14, respectively. The pharyngeal and jaw teeth became fully functional with gastric gland differentiation. The number of gastric glands and pyloric caeca and volume of the gastric blind sac increased markedly with development to the juvenile stage. Although the external morphological development of the tuna resembles the pattern of many other marine fish larvae, the basic digestive system developed at an earlier larval stage; this precocity may relate to the early appearance of piscivory and the high growth potential of tuna larvae.

Betanodaviruses, the causative agents of viral nervous necrosis in marine fish, have bipartite positive-sense RNA genomes. The viruses have been classified into 4 distinct types based on nucleotide sequence similarities in the variable region (the so-called T4 region) of the smaller genomic segment RNA2 (1.4 kb). Betanodaviruses have marked host specificity, although the primary structures of the viral RNAs and encoded proteins are similar among the viruses. We have previously demonstrated, using reassortants between striped jack nervous necrosis virus (SJNNV) and redspotted grouper nervous necrosis virus (RGNNV), that RNA2, which encodes the coat protein, strictly controls host specificity. However, because RNA2 is large, we were unable to propose a mechanism underlying this RNA2-based host specificity. To identify the RNA2 region that controls host specificity, we constructed RNA2 chimeric viruses from SJNNV and RGNNV and tested their infectivity in the original host fish, striped jack Pseudocaranx dentex and sevenband grouper Epinephelus septemfasciatus. Among these chimeric viruses, SJNNV mutants containing the variable region of RGNNV RNA2 infected sevenband grouper larvae in a manner similar to RGNNV, while RGNNV mutants containing the variable region of SJNNV RNA2 infected striped jack larvae in a manner similar to SJNNV. Immunofluorescence microscopic studies using anti-SJNNV polyclonal antibodies revealed that these chimeric viruses multiplied in the brains, spinal cords and retinas of the infected fish, as in infections by the parental viruses. These results indicate that the variable region of RNA2 is sufficient to control host specificity in SJNNV and RGNNV.

Fabry disease is an X-linked recessive inborn metabolic disorder caused by a deficiency of the lysosomal enzyme α-galactosidase A (EC 3.2. throughout the 7 exons of the α-galactosidase A gene (GLA). Mutation hotspots for Fabry disease do not exist. We examined 62 Fabry patients in Japan and found 24 GLA mutations, including 11 novel ones. A potential treatment reported for Fabry disease is active site specific chaperone (ASSC) therapy using 1-deoxygalactonojirimycin (DGJ), an inhibitor of α-galactosidase A, at subinhibitory concentrations. We transfected COS-7 cells with the 24 mutant GLAs and analyzed the α-galactosidase A activities. We then treated the transfected COS-7 cells with DGJ and analyzed its effect on the mutant enzyme activities. The activity of 11 missense mutants increased significantly with DGJ. Although ASSC therapy is useful only for misfolding mutants and therefore not applicable to all cases, it may be useful for treating many Japanese patients with Fabry disease. © 2007 Wiley-Liss, Inc.KEY WORDS: α-galactosidase A; GLA; lysosomal storage disease; 1-deoxygalactonojirimycin; chaperone therapy INTRODUCTIONFabry disease (FD; MIM# 301500) is a pan-ethnic, X-linked, lysosomal storage disorder caused by a deficiency in the lysosomal enzyme α-galactosidase A (EC 3.2.1.22) (Brady et al., 1967). Human α-galactosidase A is a homodimeric glycoprotein with three N-linked oligosaccharide chains on each subunit. Enzyme deficiency results in a systemic lysosomal accumulation of glycolipids, primarily globotriosylceramide (Gb3), in the vascular endothelium and other tissues. In the classical form of the disease, the patient develops angiokeratoma, hypohidrosis, and episodic pain crises in the extremities during childhood or adolescence. With advancing age, the morbidity of renal failure, cardiac disease, and early onset of stroke increases. The severity of the clinical manifestations depends on the amount of residual α-galactosidase A activity. Hemizygous male patients with no or very low α-galactosidase A activity usually have severe clinical symptoms and die as young adults. Heterozygous female Fabry patients exhibit a wide range of severity, from a virtually symptom-free course (Marguery et al., 1993) to one comparable to that of their male counterparts (Whybra et al., 2001), although they usually have no symptoms or very mild manifestations.We examined Fabry patients in Japan and sequenced the patients' α-galactosidase A gene (GLA) (MIM# 300644). To analyze the mutant α-galactosidase A activities, we transfected COS-7 cells with the mutant GLAs. In addition, since 1-deoxygalactonojirimycin (DGJ) stabilizes the α-galactosidase A conformation and improves its stability (Asano et al., 2000;Ishii et al., 2000;Yam et al., 2006), we added DGJ to the incubation medium and examined its effect on the mutant α-galactosidase A activities. MATERIALS AND METHODS PatientsWe examined 62 Fabry patients from 31 unrelated families. Diagnosis was based on reduced or absent α-galactosidase A activity and typical signs an...

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Yellowfin tuna Thunnus albacares larvae were reared from hatching beyond metamorphosis in May and June 1996. The larval size was 2.65mm SL at just after hatching and 27.68mm SL on Day 37. Transformation to juveniles occurred around 30 days after hatching at about 13mm SL. The larvae ini tially fed on rotifers since Day 4, on fish larvae and Artemia nauplii since Day 16, and then on frozen fish and minced fish meat. The primitive digestive system differentiated on Day 4. The gastric gland and pyloric caeca first appeared on Day 14 and 16, respectively. The pharyngeal and jaw teeth became fully functional synchronized with gastric gland differentiation. The number of gastric glands and pyloric caeca and volume of the gastric blind sac increased markedly toward the juvenile stage. Although exter nal morphological development of yellowfin tuna resembled to that of other marine fishes hatched from pelagic eggs, the digestive system developed precociously. The rapid development of the digestive sys tem allows the early appearance of piscivory which can support the high growth potential as shown in other scombrid fishes.

Broodstock management and induced spawning of flounder Paralichthys orbignyanus (Valenciennes, 1839) under a closed recirculated system Manejo de reproductores e inducción de desoves del lenguado Paralichthys orbignyanus (Valenciennes, 1839) en sistema cerrado de recirculación