It has been suggested that King George III of Great Britain suffered from the haem biosynthetic disorder, variegate porphyria. This diagnosis is pervasive throughout the scientific and popular literature, and is often referred to as the 'Royal Malady.' The authors believe it inappropriate to view the case for porphyria purely in terms of symptoms, as has generally been the case in his presumpti...

Protoporphyrinogen IX oxidase (PPO), the last common enzyme of haem and chlorophyll biosynthesis, catalyses the oxidation of protoporphyrinogen IX to protoporphyrin IX. The membrane-embedded flavoprotein is the target of a large class of herbicides. In humans, a defect in PPO is responsible for the dominantly inherited disease variegate porphyria. Here we present the crystal structure of mitoch...

1. Young SG, Fielding CJ. The ABCs of cholesterol efflux. Nat Genet 1999; 22: 316-318. 2. Fredrickson DS, Altrocchi PH, Avioli LV, Goodman DS, Goodman HC. Tangier disease. Ann Intern Med 1961; 55: 1016-1031. 3. Oram JF. Molecular basis of cholesterol homeostasis: lessons from Tangier disease and ABCA1. Trends Mol Med 2002; 8: 168-173. 4. Meissner, PN, Dailey TA, Hift RJ, et al. A R59W mutation ...

The suspected presence of hereditary disease in important historical and political figures has interested researchers for many decades. Whether Abraham Lincoln suffered from Marfan syndrome, if George III became 'mad' because he inherited variegate porphyria, and if the Romanov dynasty collapsed because the heir Alexei inherited haemophilia are important questions; physical illness can adversel...

A case of pseudoporphyria associated with excessive consumption of brewers ' yeast was studied. Detailed analysis of the yeast tablets by high performance liquid chromatography showed the presence of dicarboxylic deuteroporphyrin , mesoporphyrin, and protoporphyrin; coproporphyrin I and III isomers; and uroporphyrin I and III isomers. The faecal porphyrin concentration of the patient taking yea...

The nitrogen (N) effect of legumes is one of the main reasons for their inclusion in rotation systems and their success in rainfed agriculture of Mediterranean areas. The comparative analysis of this effect in relation to alternative systems or practices is essential for a comprehensive appreciation in their merit. This field experiment was comprised of four three-year rotation cycles. Wheat (T...

The symbiotic partnerships between legumes and their root-nodule bacteria (rhizobia) vary widely in their degree of specificity, but the underlying reasons are not understood. To assess the potential for host-range evolution, we have investigated microheterogeneity among the shared symbionts of a group of related legume species. Host specificity and genetic diversity were characterized for a so...

Legume crops are known to have low soil N uptake early in their life cycle, which can weaken their ability to compete with other species, such as weeds or other crops in intercropping systems. However, there is limited knowledge on the main traits involved in soil N uptake during early growth and for a range of species. The objective of this research was to identify the main traits explaining t...

Protein kinases are involved in signal transduction for environmental stress responses. In response to drought and salinity, a 48-kDa protein kinase (AAPK; abscisic acid-activated protein kinase (AAPK) in guard cells is activated by abscisic acid (ABA) and phosphorylates several targets such as the carboxy-terminus of inward-rectifying K+ channel and heterogeneous mRNA binding protein to adopt ...

Maximum concentrations of microsomal cytochrome P-450 are present in 3-4 day-old mung beans (Phaseolus aureus). On illumination of dark-grown seedlings, cytochrome P-450 and later cytochrome P-450 undergo a rapid decrease in concentration in vivo, with an apparent half-time of about 6 h. Conversely light-grown seedlings, transferred to darkness, show a slow accumulation of cytochrome P-450, dou...