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931.
Lambrechts D Storkebaum E Morimoto M Del-Favero J Desmet F Marklund SL Wyns S Thijs V Andersson J van Marion I Al-Chalabi A Bornes S Musson R Hansen V Beckman L Adolfsson R Pall HS Prats H Vermeire S Rutgeerts P Katayama S Awata T Leigh N Lang-Lazdunski L Dewerchin M Shaw C Moons L Vlietinck R Morrison KE Robberecht W Van Broeckhoven C Collen D Andersen PM Carmeliet P 《Nature genetics》2003,34(4):383-394
932.
Mutations in a Sar1 GTPase of COPII vesicles are associated with lipid absorption disorders 总被引:10,自引:0,他引:10
Jones B Jones EL Bonney SA Patel HN Mensenkamp AR Eichenbaum-Voline S Rudling M Myrdal U Annesi G Naik S Meadows N Quattrone A Islam SA Naoumova RP Angelin B Infante R Levy E Roy CC Freemont PS Scott J Shoulders CC 《Nature genetics》2003,34(1):29-31
Dietary fat is an important source of nutrition. Here we identify eight mutations in SARA2 that are associated with three severe disorders of fat malabsorption. The Sar1 family of proteins initiates the intracellular transport of proteins in COPII (coat protein)-coated vesicles. Our data suggest that chylomicrons, which vastly exceed the size of typical COPII vesicles, are selectively recruited by the COPII machinery for transport through the secretory pathways of the cell. 相似文献
933.
Mutations in the genes encoding 11beta-hydroxysteroid dehydrogenase type 1 and hexose-6-phosphate dehydrogenase interact to cause cortisone reductase deficiency 总被引:12,自引:0,他引:12
934.
Effects of household dynamics on resource consumption and biodiversity 总被引:41,自引:0,他引:41
Human population size and growth rate are often considered important drivers of biodiversity loss, whereas household dynamics are usually neglected. Aggregate demographic statistics may mask substantial changes in the size and number of households, and their effects on biodiversity. Household dynamics influence per capita consumption and thus biodiversity through, for example, consumption of wood for fuel, habitat alteration for home building and associated activities, and greenhouse gas emissions. Here we report that growth in household numbers globally, and particularly in countries with biodiversity hotspots (areas rich in endemic species and threatened by human activities), was more rapid than aggregate population growth between 1985 and 2000. Even when population size declined, the number of households increased substantially. Had the average household size (that is, the number of occupants) remained static, there would have been 155 million fewer households in hotspot countries in 2000. Reduction in average household size alone will add a projected 233 million additional households to hotspot countries during the period 2000-15. Rapid increase in household numbers, often manifested as urban sprawl, and resultant higher per capita resource consumption in smaller households pose serious challenges to biodiversity conservation. 相似文献
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Fresnel lenses allow almost perfect imaging in widely different circumstances, but their focus is perfect only for a single wavelength. Wang et al. have shown how the effective bandpass may be widened for X-ray microscopy by using a compound diffractive/refractive lens near to an absorption edge. A compound lens has also been proposed for high-energy astronomy, working well above all absorption edges. Although the scale is very different, we point out here that the principle is the same. Ever since Galileo constructed an astronomical telescope that he was able to reconfigure to study fleas and gnats, astronomy and microscopy have relied on optics that are closely related, but different in detail. 相似文献
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Group I metabotropic glutamate receptors (consisting of mGluR1 and mGluR5) are G-protein-coupled neurotransmitter receptors that are found in the perisynaptic region of the postsynaptic membrane. These receptors are not activated by single synaptic volleys but rather require bursts of activity. They are implicated in many forms of neural plasticity including hippocampal long-term potentiation and depression, cerebellar long-term depression, associative learning, and cocaine addiction. When activated, group I mGluRs engage two G-protein-dependent signalling mechanisms: stimulation of phospholipase C and activation of an unidentified, mixed-cation excitatory postsynaptic conductance (EPSC), displaying slow activation, in the plasma membrane. Here we report that the mGluR1-evoked slow EPSC is mediated by the TRPC1 cation channel. TRPC1 is expressed in perisynaptic regions of the cerebellar parallel fibre-Purkinje cell synapse and is physically associated with mGluR1. Manipulations that interfere with TRPC1 block the mGluR1-evoked slow EPSC in Purkinje cells; however, fast transmission mediated by AMPA-type glutamate receptors remains unaffected. Furthermore, co-expression of mGluR1 and TRPC1 in a heterologous system reconstituted a mGluR1-evoked conductance that closely resembles the slow EPSC in Purkinje cells. 相似文献