Acute intermittent porphyria caused by a C→T mutation that produces a stop codon in the porphobilinogen deaminase gene

Summary A mutation of the porphobilinogen (PBG) deaminase gene that produces the cross-reacting immunological material (CRIM)-negative type of acute intermittent porphyria (AIP) has been identified in one of 43 unrelated patients with this form of the disorder. The mutation is a CT transition that abolishes a PstI recognition site in exon 9 of the gene and converts a codon for glutamine to a stop codon.     

FREE-FLOW ELECTROPHORETIC SEPARATION AND ELECTRICAL SURFACE PROPERTIES OF SUBCELLULAR PARTICLES FROM GUINEA PIG BRAIN

Continuous free-flow electrophoretic separation has been used to obtain relatively pure preparations of synaptosomes and synaptic vesicles from crude fractions of guinea pig brain homogenates. Measurements of the contents of protein, neuraminic acid, and bound acetylcholine; the activities of succinic dehydrogenase, adenosine triphosphatase, choline acetylase, and 5'-nucleotidase; and the uptake of ¹⁴C-labeled choline arid acetylcholine in the presence and absence of hemicholinium, all confirm the electron microscope evidence that the electrophoretic preparations are at least as pure as those obtained by ultracentrifugal methods. The electrophoretic mobility measurements have been used to calculate zeta potentials and surface charge densities for these particles.     

DETERMINATION OF GUSTATORY SUCROSE THRESHOLD IN WATER BY USE OF A LINEAR SUCROSE GRADIENT

A novel experimental method was developed which allows the determination of the threshold concentration of sucrose by use of a linear sucrose gradient in water. With this method a continuous tasting of the test-liquid is possible. A panel of 15 persons experienced in taste-testing was used. Three gradients of different steepness were applied: 0 to 1.5% (w/w) sucrose in 2 min (I), 3 min (II) and 4 min (III). The results of the new method were compared with those of the standard method (DIN). With gradients I and II we found values which were significantly higher than those of the standard method (I: 0.49% (w/w); II: 0.46% (w/w); DIN: 0.31% (w/w)), whereas with gradient III the same threshold value was found as with the DIN-Method (III: 0.32% (w/w)).     

Quantification of the permeability of the blood-CSF barrier to alpha-MSH in the rat

The ability of alpha-MSH to cross the blood-CSF barrier of the rat was assessed by measurement of the rate of appearance of immunoreactive alpha-MSH in a cerebrospinal fluid (CSF) perfusate following intravenous injection of peptide. Comparisons were made with the rate of appearance of a simultaneously administered dose of 14C-inulin which is poorly permeable at the blood-CSF barrier. Concentrations of drugs measured in plasma were fitted to two-compartment pharmacokinetic models, and those measured in the CSF perfusate to one-compartment open systems receiving an input from the plasma compartment. The rate constant for entry of alpha-MSH into CSF was 0.00087 min-1, which was not significantly different from that for inulin of 0.00055 min-1. As alpha-MSH penetrated into CSF at a rate comparable to inulin, it was concluded that the limited entry of peptide was by aqueous diffusion along with other water-soluble macromolecules.     

The biology and energetics of the potato aphid Macrosiphum euphorbiae, living in galls of the apple aphids Dysaphis devecta and Aphis pomi

A non-gall-forming aphid, Macrosiphum euphorbiae, was reared within the galls of Dysaphis devecta and Aphis pomi which had been induced in apple seedlings. Similar aphids were also reared on ungalled seedling leaves of a similar age and also on mature leaves and in old galls.There is a weak positive linear relationship between lipid content and pre-reproductive adult dry weight, but aphids living on young galled or ungalled plant tissue have 30% lipid compared to 11% for aphids living on old plant tissue. Adult aphids gradually decline in weight, losing between 24% and 38% of their pre-reproductive biomass before death. The energy content of this loss subsidieses reproduction. Reproduction of aphids feeding in old galls or on mature leaves was negligible, but on younger tissues average fecundity was 31, there being little difference between aphids living on galled and ungalled tissue. There is no simple relationship between pre-reproductive adult embryo content and fecundity.The principal factor mediating honeydew production was aphid size and this factor overrides any variation caused by different feeding sites. Honeydew production averaged 0.43 mg during a life of 30 days. Aphids successfully completing their life cycle have a life-time energy consumption of 30.58 joules of which 18% is lost as heat during respiration (R), 30% is contained in honeydew (F+U), 1% is contained in exuviae (Pe). Larval growth accounts for 17% (Pg), and adult reproduction for 34% (Pr).The P/C ratio for M. euphorbiae is 53% and is broadly comparable with aphids living on herbaceous plants.

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Résumé Un puceron ne formant pas de galles, Macrosiphum euphorbiae, a été élevé dans des galles de Dysaphis devecta et Aphis pomi formées sur semis de pommiers. D'autres M. euphorbiae ont été élevés sur feuilles sans galles du même âge provenent de semis identiques, sur feuilles mûres et sur vieilles galles.Il y a une faible relation positive linéaire entre la teneur en lipides et le poids sec des adultes avant la reproduction, mais les pucerons élevés sur tissus végétaux jeunes avec ou sans galles contiennent 30% de lipides contre 11% pour ceux élevés sur tissus âgés. Le poids des pucerons adultes diminue progressivement, la perte avant la mort se situant entre 24 et 38% de la biomasse avant la reproduction. Le contenu énergétique de ces pertes alimente la reproduction. La reproduction de pucerons consommant les vieilles galles ou les feuilles mûres était négligeable, mais sur jeunes tissus la fécondité moyenne était de 31-avec peu de différences entre pucerons sur galles ou feuilles-. Il n'y a pas de corrélation simple entre le contenu en embryon des adultes avant la reproduction et la fécondité.Le principal paramètre conditionnant la production de miellat est la taille du puceron; ce paramètre surpasse toute variation provoquée par les divers lieux d'alimentation. La production moyenne de miellat est de 0,43-mg pendant une vie de 30 jours. Les pucerons accomplissant leur cycle avec succès utilisent pendant leur vie 30,58 joules, dont 18% sont dissipés sous forme de chaleur pendant la respiration (R); le contenu du miellat (F+U) correspond à 30%, et 1% est contenu dans l'exuvie (Pe). La croissance larvaire (Pg) utilise 17%, et l'activité reproductrice des adultes (Pr) 34% de l'énergie. Le rapport P/C-53% pour M. euphorbiae-est globalement comparable à celui des pucerons vivant sur plantes herbacées.