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41.
Battin TJ  Kaplan LA  Denis Newbold J  Hansen CM 《Nature》2003,426(6965):439-442
In many aquatic ecosystems, most microbes live in matrix-enclosed biofilms and contribute substantially to energy flow and nutrient cycling. Little is known, however, about the coupling of structure and dynamics of these biofilms to ecosystem function. Here we show that microbial biofilms changed the physical and chemical microhabitat and contributed to ecosystem processes in 30-m-long stream mesocosms. Biofilm growth increased hydrodynamic transient storage-streamwater detained in quiescent zones, which is a major physical template for ecological processes in streams-by 300% and the retention of suspended particles by 120%. In addition, by enhancing the relative uptake of organic molecules of lower bioavailability, the interplay of biofilm microarchitecture and mass transfer changed their downstream linkage. As living zones of transient storage, biofilms bring hydrodynamic retention and biochemical processing into close spatial proximity and influence biogeochemical processes and patterns in streams. Thus, biofilms are highly efficient and successful ecological communities that may also contribute to the influence that headwater streams have on rivers, estuaries and even oceans through longitudinal linkages of local biogeochemical and hydrodynamic processes.  相似文献   
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Sensory motor neuropathy is associated with various inherited disorders including Charcot-Marie-Tooth disease, X-linked adrenoleukodystrophy/adrenomyeloneuropathy and Refsum disease. In the latter two, the neuropathy is thought to result from the accumulation of specific fatty acids. We describe here three patients with elevated plasma concentrations of pristanic acid (a branched-chain fatty acid) and C27-bile-acid intermediates. Two of the patients suffered from adult-onset sensory motor neuropathy. One patient also had pigmentary retinopathy, suggesting Refsum disease, whereas the other patient had upper motor neuron signs in the legs, suggesting adrenomyeloneuropathy. The third patient was a child without neuropathy. In all three patients we discovered a deficiency of alpha-methylacyl-CoA racemase (AMACR). This enzyme is responsible for the conversion of pristanoyl-CoA and C27-bile acyl-CoAs to their (S)-stereoisomers, which are the only stereoisomers that can be degraded via peroxisomal beta-oxidation. Sequence analysis of AMACR cDNA from the patients identified two different mutations that are likely to cause disease, based on analysis in Escherichia coli. Our findings have implications for the diagnosis of adult-onset neuropathies of unknown aetiology.  相似文献   
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Autosomal recessive spastic ataxia of Charlevoix-Saguenay (ARSACS or SACS) is an early onset neurodegenerative disease with high prevalence (carrier frequency 1/22) in the Charlevoix-Saguenay-Lac-Saint-Jean (CSLSJ) region of Quebec. We previously mapped the gene responsible for ARSACS to chromosome 13q11 and identified two ancestral haplotypes. Here we report the cloning of this gene, SACS, which encodes the protein sacsin. The ORF of SACS is 11,487 bp and is encoded by a single gigantic exon spanning 12,794 bp. This exon is the largest to be identified in any vertebrate organism. The ORF is conserved in human and mouse. The putative protein contains three large segments with sequence similarity to each other and to the predicted protein of an Arabidopsis thaliana ORF. The presence of heat-shock domains suggests a function for sacsin in chaperone-mediated protein folding. SACS is expressed in a variety of tissues, including the central nervous system. We identified two SACSmutations in ARSACS families that lead to protein truncation, consistent with haplotype analysis.  相似文献   
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L Lefran?ois  M J Bevan 《Nature》1985,314(6010):449-452
Plasma membrane glycoproteins of cytotoxic T lymphocytes (CTLs) are involved in the binding to and subsequent destruction of appropriate target cells. The electrophoretic profile of surface proteins of mature CTLs, particularly those of high relative molecular mass (Mr), is markedly different from that of naive peripheral T cells or non-cytolytic T cells, suggesting the possible involvement of these molecules in the activation of CTLs and/or in the lytic process itself. By generating monoclonal antibodies to cell-surface proteins of CTL clones, we have now detected CTL-specific modifications in one of these high-Mr membrane proteins, T200. Although forms of T200 are found on a wide variety of cell types, the neoantigenic determinants recognized by our antibodies are present exclusively on activated T cells and in high concentrations only on CTLs. Furthermore, the expression of the modifications recognized by our antibodies is influenced by soluble factors and also seems to have functional significance, as monoclonal antibodies specific for these novel epitopes block cytolytic activity.  相似文献   
48.
Pro-Latour     
In this comment I want to clarify five topics. The first topic concerns the importance of looking back at the very principles of the foundations of Western society. The second comment argues for the original position of Latour within the field of (social) constructivism. In the third comment, I argue that Haraway adds to the science-politics discussion by elaborating her philosophy beyond dichotomy. In the fourth comment, I argue that the terms ‘objective’ and ‘rational’ are central philosophical concepts which should be retained. Finally I will make the connection between ‘what’ is represented and ‘how’ to represent it.  相似文献   
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Summary By using paper electrophoresis, the authors succeeded in demonstrating different protein fractions in human and animal lenses.Qualitative and quantitative differences were noted in different animals, between the cortical and the nuclear areas of the lens, and also according to age.

Chargé de Recherches du Fonds national de la Recherche scientifique.  相似文献   
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