The older literature concerned with cystinuria has been reviewed in detail frequently", 21, 38 44. It is, therefore, the purpose of this review to discuss the more recent literature only, and we have chosen to discuss mainly the literature of the past ten years, the period which hlas elapsed since the most recent revision of Garrod's able presentation of the subject"3. Cystinuria, discovered in...

The study of the proximal tubular function in inherited renal Fanconi syndrome offers a unique opportunity to augment insights in proximal tubular transport and signalling pathways and allows a better interpretation of tests applied for the evaluation of proximal tubular function in acquired disorders. The causes of inherited and acquired renal Fanconi syndrome are listed in Table 1. It is a co...

We identify three dimeric proteins whose mechanostability is anisotropic and should exceed 1 nN along some directions. They come with distinct mechanical clamps: either shear-based, or involving a cystine slipknot, or due to dragging of a cystine plug through a cystine ring. The latter two mechanisms are topological in nature; the cystine plug mechanism has not yet been discussed but it turns o...

Cystinosis is a rare disorder, and, accordingly, progress on the understanding and treatment of this disease has been relatively slow. Although cystinosis was identified over 100 years ago, the history of cystinosis is marked by a few sudden leaps forward in our understanding rather than by a sustained research effort fuelled by the larger research community. Major conceptual break-throughs inc...

The stimulatory effect of GSSG on the reduction of cytochrome c by GSH has been shown to be due to SO-containing impurities; GSSG itself has no stimulatory effect. Methods are described for the production of such impurities in high yield. Similar effects are shown by cystine trisulfide. The stimulation of the cytochrome c reduction rate has been found to be catalytic; one molecule of cystine tr...

Four hundred and seventy-eight pregnant women attending local authority clinics in Portsmouth were screened for bacteriuria using the dip-inoculum transport medium (DITM) spoon method. The detection of asymptomatic bacteriuria in 5% of the patients with a very low proportion of equivocal results (0.83%) suggests that this is an efficient method; and large numbers of urine specimens could be sam...

We consider mechanical stability of dimeric and monomeric proteins with the cystine knot motif. A structure based dynamical model is used to demonstrate that all dimeric and some monomeric proteins of this kind should have considerable resistance to stretching that is significantly larger than that of titin. The mechanisms of the large mechanostability are elucidated. In most cases, it originat...

Tanjungides A (1) (Z isomer) and B (2) (E isomer), two novel dibrominated indole enamides, have been isolated from the tunicate Diazona cf formosa. Their structures were determined by spectroscopic methods including HRMS, and extensive 1D and 2D NMR. The stereochemistry of the cyclised cystine present in both compounds was determined by Marfey's analysis after chemical degradation and hydrolysi...

Cystinosis is an autosomal recessive inherited disease that causes disfunction in many organs. The aim of this study is to evaluate the results obtained by applying treatment through a frequency modulation device (bicom bioresonance) in a cystinosis case every week for four years. The study was conducted in a private acupuncture clinic in Eskisehir, Turkey. In the study, the case is firstly pre...