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11.
Cisca Wijmenga SaraT. Winokur GeorgeW. Padberg Mette I. Skraastad Michael R. Altherr John J. Wasmuth Jeffrey C. Murray Marten H. Hofker Rune R. Frants 《Human genetics》1993,92(2):198-203
Facioscapulohumeral muscular dystrophy (FSHD) is a relatively common autosomal dominant neuromuscular disorder. The gene for FSHD has recently been assigned to chromosome 4q35. Although abnormal mitochondrial and biochemical changes have been observed in FSHD, the molecular defect is unknown. In addition to the FSHD gene, the human muscle adenine nucleotide translocator gene (ANT1) is located on chromosome 4. Interestingly, biochemical studies recently showed a possible defect of ANT1. In order to evaluate the potential role of ANT1 in the etiology of FSHD, the human ANT1 gene was isolated by cosmid cloning and localized to 4q35, in the region containing the FSHD gene. However, in situ hybridization and physical mapping of somatic cell hybrids localized the ANT1 gene proximal to the FSHD gene. In addition, a polymorphic CA-repeat 5 kb upsstream of the ANT1 gene was used as a marker in FSHD and Centre d'Etude du Polymorphisme Humain families to perform linkage analysis. These data together exclude ANT1 as the primary candidate gene for FSHD. The most likely order of the loci on chromosome 4q35 is cen-ANT1-D4S171-F11-D4S187-D4S163-D4S139-FSHD-tel. 相似文献
12.
J. Jelle Lever Egbert H. Van Nes Marten Scheffer Jordi Bascompte 《Ecology letters》2023,26(10):1765-1779
Theory suggests that increasingly long, negative feedback loops of many interacting species may destabilize food webs as complexity increases. Less attention has, however, been paid to the specific ways in which these ‘delayed negative feedbacks’ may affect the response of complex ecosystems to global environmental change. Here, we describe five fundamental ways in which these feedbacks might pave the way for abrupt, large-scale transitions and species losses. By combining topological and bioenergetic models, we then proceed by showing that the likelihood of such transitions increases with the number of interacting species and/or when the combined effects of stabilizing network patterns approach the minimum required for stable coexistence. Our findings thus shift the question from the classical question of what makes complex, unaltered ecosystems stable to whether the effects of, known and unknown, stabilizing food-web patterns are sufficient to prevent abrupt, large-scale transitions under global environmental change. 相似文献
13.
It is usually thought that unlike terrestrial plants, phytoplankton will not show a significant response to an increase of atmospheric CO2. Here we suggest that this view may be biased by a neglect of the effects of carbon (C) assimilation on the pH and the dissociation of the C species. We show that under eutrophic conditions, productivity may double as a result of doubling of the atmospheric CO2 concentration. Although in practice productivity increase will usually be less, we still predict a productivity increase of up to 40% in marine species with a low affinity for bicarbonate. In eutrophic freshwater systems doubling of atmospheric CO2 may result in an increase of the productivity of more than 50%. Freshwaters with low alkalinity appeared to be very sensitive to atmospheric CO2 elevation. Our results suggest that the aquatic C sink may increase more than expected, and that nuisance phytoplankton blooms may be aggravated at elevated atmospheric CO2 concentrations. 相似文献
14.
The prokaryote Cyanocyta korschikoffiana was isolated from the eukaryote Cyanophora paradoxa. The synthesis of several thylakoid proteins in these cyanelles is influenced by light and darkness and is sensitive to cycloheximide, the inhibitor of the eukaryotic host's translation. The possibility of a direct coordination between the translations of the host and of the cyanelles is discussed.Abbreviations CHM treatment
addition of cycloheximide
- CPN
chlorophylline
- PBN
phycobiline
- SDS-PAGE
sodium-dodecylsulphate-polyacrylamide gelelectrophoresis 相似文献
15.
Gert Rijksen Gerard E. J. Staal Margreet J. M. van der Vlist Frits A. Beemer Jaap Troost Wolf Gutensohn Jan P. R. M. van Laarhoven Chris H. M. M. de Bruyn 《Human genetics》1981,57(1):39-47
Summary A patient with the full clinical expression of the classical Lesch-Nyhan syndrome is presented with a residual hypoxanthine-guanine phosphoribosyl transferase (HGPRT) activity of 5–10% in erythrocyte lysate and about 30% in fibroblast lysate. The activities of other erythrocyte enzymes of purine metabolism were typical for a classical Lesch-Nyhan patient. The effects of allopurinol therapy on the excretion of urinary purine metabolites were studied by a newly developed isotachophoretic technique.The unusually high residual activity of HGPRT in erythrodytes and fibroblasts of the patient enabled the enzymologic characterization of the mutant enzyme: in fibroblasts the affinities for the substrates hypoxanthine and guanine were normal. However, there was an increased apparent K
m for phosphoribosylpyrophosphate (PRPP), a complete absence of product inhibition by IMP and GMP, and a decreased heat stability. Addition of PRPP did not stabilize the mutant enzyme. In addition to the altered properties of the fibroblast enzyme, the K
m of the erythrocyte enzyme for hypoxanthine was also increased.Immunoprecipitation experiments revealed the presence of an approximately normal amount of material cross-reacting with anti-human HGPRT antiserum. However, it appeared that this cross-reacting material had a decreased stability. When intact erythrocytes were incubated with radiolabeled purine bases, no formation of IMP or GMP could be detected, despite the relatively high residual activity of HGPRT in the hemolysate. The results fit the following hypothesis: as a consequence of a structural mutation affecting the PRPP-site of the enzyme and a decreased heat stability, the activity of the mutant enzyme under in vivo conditions is virtually zero.In the erythrocytes of the patient's mother a normal HGPRT-activity was found. However, the activity in her fibroblasts was lower than normal, while a decreased heat stability and an intermediate behavior towards IMP could be shown.Hair root analysis of several members of the patient's family confirmed the heterozygosity of the mother, whereas no other heterozygotes could be detected. The family anamnesis did not show other cases of Lesch-Nyhan syndrome. These findings were taken as evidence that the patient described in this paper might represent a mutation orginating from the gametes in either of the maternal grandparents. 相似文献
16.
Michael Marten 《Hydrobiologia》1990,194(3):199-201
A water-bath-system which simultaneously provides eight different temperatures for rearing aquatic organisms is presented.
Temperatures ranged from 4 to 18 °C, at 2 ± 0.3 °C intervals. It has an useful area of c. 1 m2, but can be adapted easily to any specific requirement. It is inexpensive (c. U.S.$1200), requiring only simple technical
equipment. 相似文献
17.
A new variant of glucosephosphate isomerase deficiency. 总被引:1,自引:0,他引:1
J P Van Biervliet A Vlug H Bartstra J J Rotteveel G A de Vaan G E Staal 《Humangenetik》1975,30(1):35-40
A new variant of glucose-6-phosphate isomerase deficiency is described. The enzyme kinetics and properties were studied. Genetic and electrophoretic data pointed to a double heterozygous state in the patient. These data are compared to the other variants described in the literature until now. 相似文献
18.
Paul Sroka Robert H. Barth Lawrence I. Gilbert Gerardus B. Staal 《Journal of insect physiology》1975,21(2):463-469
Exogenously applied natural juvenile hormones (JH) and some synthetic JH mimetic substances resulted in dose-related gonadotropic responses and were able to fully substitute for the absence of endogenous JH in a moth and a cockroach. Aliphatic methyl and ethyl esters, thio esters, and amides were most active. Some aromatic JH mimics with high activity in morphogenetic assays on a variety of insects showed little activity in the gonadotropic bioassay. This suggests that the ovaries of the moth Manduca sexta and the cockroach Nauphoeta cinerea respond primarily to the intrinsic JH activity of the test substances. 相似文献
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