Excessive quantities of amino-acids have recently been found in the urine of infants with galactosaemia (Holzel, Komrower and Wilson, 1952; Bickel and Hickmans, 1952). At first sight, the occurrence of amino-aciduria in a condition known to be associated with severe liver damage would suggest that the amino-aciduria is of the 'overflow' variety, namely, that it occurs as the result of a rise of...

Hydrogen sulfide (sulfide, H(2)S) is a colorless, water-soluble gas with a typical smell of rotten eggs. In the past, it has been investigated for its role as a potent toxic gas emanating from sewers and swamps or as a by-product of industrial processes. At high concentrations, H(2)S is a powerful inhibitor of cytochrome c oxidase; in trace amounts, it is an important signaling molecule, like n...

True positive result denotes correct placement of hemodialysis catheter according to bubble-enhanced ultrasound and chest radiography. True negative result 1⁄4 incorrect placement of hemodialysis catheter according to bubble-enhanced ultrasound and chest radiography. False positive result 1⁄4 correct placement of hemodialysis catheter according to bubble-enhanced ultrasound not confirmed by che...

Methylmalonic aciduria (MMA) is a disorder of organic acid metabolism resulting from a functional defect of methylmalonyl-CoA mutase (MCM). MMA is associated with significant morbidity and mortality, thus therapies are necessary to help improve quality of life and prevent renal and neurological complications. Transgenic mice carrying an intact human MCM locus have been produced. Four separate t...

Minor products in the reaction between substituted 1,3-diiminoisoindolines and 2,5-diamino-3,4-dicyanothiophene were identified as the nickel seco-tribenzoporphyrazines 4 and 5, which have been characterized by UV-vis, MCD, NMR, and mass spectroscopy. Experimentally observed tetraazachlorin-type UV-vis spectra of new seco-tribenzoporphyrazines were explained on the basis of DFT and TDDFT calcul...