Mutations in a human homologue of Drosophila crumbs cause retinitis pigmentosa (RP12).

Retinitis pigmentosa (RP) comprises a clinically and genetically heterogeneous group of diseases that afflicts approximately 1.5 million people worldwide. Affected individuals suffer from a progressive degeneration of the photoreceptors, eventually resulting in severe visual impairment. To isolate candidate genes for chorioretinal diseases, we cloned cDNAs specifically or preferentially expressed in the human retina and the retinal pigment epithelium (RPE) through a novel suppression subtractive hybridization (SSH) method. One of these cDNAs (RET3C11) mapped to chromosome 1q31-q32.1, a region harbouring a gene involved in a severe form of autosomal recessive RP characterized by a typical preservation of the para-arteriolar RPE (RP12; ref. 3). The full-length cDNA encodes an extracellular protein with 19 EGF-like domains, 3 laminin A G-like domains and a C-type lectin domain. This protein is homologous to the Drosophila melanogaster protein crumbs (CRB), and denoted CRB1 (crumbs homologue 1). In ten unrelated RP patients with preserved para-arteriolar RPE, we identified a homozygous AluY insertion disrupting the ORF, five homozygous missense mutations and four compound heterozygous mutations in CRB1. The similarity to CRB suggests a role for CRB1 in cell-cell interaction and possibly in the maintenance of cell polarity in the retina. The distinct RPE abnormalities observed in RP12 patients suggest that CRB1 mutations trigger a novel mechanism of photoreceptor degeneration.     

An Experimental Study of Aspect Ratio on the Tensile Properties of an Elastic Fabric for Making-up of Pressure Garments

The“cut-and-sewn” pressure garments are normally tailored made in various sizes according to the size of human body as well as the area of burn wounds.When a tubular pressure garment is cut in different length and width measurements,different aspect ratio will be occurred on the elastic fabric for making up the pressuregarment. Many therapists in Hong Kong concerned the change of aspect ratio may affect the tensile properties of the elastic fabric and ultimately will affect the skin-and-garment interface pressure for the patient.The aim of     

测定纱线弯曲刚度的实验方法

为了研究织机上的打纬和织物形成过程,本文提出了以频闪摄影测定纱线弯曲刚度的实验方法,并运用该方法对不同纱线的弯曲刚度进行了测定。     

Regulation of sodium and body fluid homeostasis during development: Implications for the pathogenesis of hypertension

The spontaneously hypertensive rat (SHR) is an important animal model of human essential hypertension. During the first month of life, increased retention of sodium is present in the SHR which appears to be mediated by the renin-angiotensin system. The present review will discuss the role that increased activity of the renin-angiotensin system plays in sodium/body fluid regulation during early development. It is hypothesized that disordered regulation of sodium/body fluid homeostasis during this stage leads to pathological cardiovascular regulation in adulthood. Through an understanding of the relationship between sodium/body fluid balance in the young and cardiovascular function in the adult insights may be gained into both the pathological state of hypertension and the critical role played by early development in shaping homeostatic mechanisms in adulthood.     

Trail-following responses ofLeptogenys diminuta to stereoisomers of 4-methyl-3-heptanol

Behavioral tests carried out with the four stereoisomers of 4-methyl-3-heptanol revealed thatLeptogenys diminuta ants respond specifically only to the (3R, 4S)-isomer.     

Identification of 2-deoxyecdysone from the mite,Tyrophagus putrescentiae

2-Deoxyecdysone was purified from the miteTyrophagus putrescentiae and monitored by a radioimmunoassay. The compound was identified by GC-MS and LC-MS. This is the first report of an endogenous ecdysteroid from a mite.     

A potent attractant of zoospores ofAphanomyces cochlioides isolated from its host,Spinacia oleracea

A highly potent attractant of zoospores ofAphanomyces cochlioides, a causal fungus of the root rot disease of spinach (Spinacia oleracea), was isolated from spinach roots, and its structure was determined by spectroscopic evidence and chemical synthesis as cochliophilin A (5-hydroxy-6,7-methylenedioxyflavone,1). A chromosorb particle prepared by soaking in solution of1 showed a potent attracting activity toward the zoospores using concentrations of1 above 10^–9 or 10^–10 M.     

Gastrointestinal transit and digestibility of maltitol,sucrose and sorbitol in rats: A multicompartmental model and recovery study

Using data obtained with a dye marker and the gavage technique, the kinetics of gastrointestinal transit of different loads of sugar substitutes (maltitol, sorbitol) and sugar (sucrose) in the rat were analysed using a linear multicompartmental model over a range from the realistic to the non-physiologic high, of carbohydrate intake levels and using only a few experimental time points. The model gave detailed insight into intestinal propulsion and gastrocecal transit time. Rate constants of transport between the compartments investigated were determined; they showed characteristics which could be related to the substance and the dosage administered. Analyses of the gastrointestinal content and calculations of the intestinal net water movement showed that the digestibility and absorption of the disaccharide sugar alcohol, maltitol, in the small gut depended inversely on the dose ingested. For all substances tested, caloric availability in the small intestine was calculated. At a physiological low level of maltitol intake, the results also indicated an insignificant calorie-saving effect in comparison to sucrose, an effect based mainly on the slow absorption rate of the maltitol cleavage product sorbitol.     

Multiplication of human-derivedPneumocystis carinii in severe combined immunodeficient (SCID) mice

clinically healthy SCID mice were infected intratracheally withPneumocystis carinii (PC) of human origin. The data obtained provides unambiguous evidence that progressive multiplication of PC organisms of human origin takes place in the lungs of experimentally infected animals. SCID mice that were infected with human-derived PC also revealed a markedly greater number of mouse PC organisms in their lungs than the controls. All the SCID recipients of human PC died by day 65 post infection, whereas the controls, housed under identical conditions, started dying significantly later due to severe mouse pneumocystosis. This animal model could be used for the maintenance and propagation of human PC, and for evaluating strategies for treating human pneumocystosis.