排序方式: 共有164条查询结果,搜索用时 31 毫秒
91.
Laser ablation inductively coupled plasma mass spectrometry imaging of metals in experimental and clinical Wilson's disease
下载免费PDF全文
![点击此处可从《Journal of cellular and molecular medicine》网站下载免费的PDF全文](/ch/ext_images/free.gif)
Sorina Georgiana Boaru Uta Merle Ricarda Uerlings Astrid Zimmermann Christa Flechtenmacher Claudia Willheim Elisabeth Eder Peter Ferenci Wolfgang Stremmel Ralf Weiskirchen 《Journal of cellular and molecular medicine》2015,19(4):806-814
Wilson's disease is an autosomal recessive disorder in which the liver does not properly release copper into bile, resulting in prominent copper accumulation in various tissues. Affected patients suffer from hepatic disorders and severe neurological defects. Experimental studies in mutant mice in which the copper‐transporting ATPase gene (Atp7b) is disrupted revealed a drastic, time‐dependent accumulation of hepatic copper that is accompanied by formation of regenerative nodes resembling cirrhosis. Therefore, these mice represent an excellent exploratory model for Wilson's disease. However, the precise time course in hepatic copper accumulation and its impact on other trace metals within the liver is yet poorly understood. We have recently established novel laser ablation inductively coupled plasma mass spectrometry protocols allowing quantitative metal imaging in human and murine liver tissue with high sensitivity, spatial resolution, specificity and quantification ability. By use of these techniques, we here aimed to comparatively analyse hepatic metal content in wild‐type and Atp7b deficient mice during ageing. We demonstrate that the age‐dependent accumulation of hepatic copper is strictly associated with a simultaneous increase in iron and zinc, while the intrahepatic concentration and distribution of other metals or metalloids is not affected. The same findings were obtained in well‐defined human liver samples that were obtained from patients suffering from Wilson's disease. We conclude that in Wilson's disease the imbalances of hepatic copper during ageing are closely correlated with alterations in intrahepatic iron and zinc content. 相似文献
92.
James W. Horn Zhenxiang Xi Ricarda Riina Jess A. Peirson Ya Yang Brian L. Dorsey Paul E. Berry Charles C. Davis Kenneth J. Wurdack 《Evolution; international journal of organic evolution》2014,68(12):3485-3504
The mid‐Cenozoic decline of atmospheric CO2 levels that promoted global climate change was critical to shaping contemporary arid ecosystems. Within angiosperms, two CO2‐concentrating mechanisms (CCMs)—crassulacean acid metabolism (CAM) and C4—evolved from the C3 photosynthetic pathway, enabling more efficient whole‐plant function in such environments. Many angiosperm clades with CCMs are thought to have diversified rapidly due to Miocene aridification, but links between this climate change, CCM evolution, and increased net diversification rates (r) remain to be further understood. Euphorbia (~2000 species) includes a diversity of CAM‐using stem succulents, plus a single species‐rich C4 subclade. We used ancestral state reconstructions with a dated molecular phylogeny to reveal that CCMs independently evolved 17–22 times in Euphorbia, principally from the Miocene onwards. Analyses assessing among‐lineage variation in r identified eight Euphorbia subclades with significantly increased r, six of which have a close temporal relationship with a lineage‐corresponding CCM origin. Our trait‐dependent diversification analysis indicated that r of Euphorbia CCM lineages is approximately threefold greater than C3 lineages. Overall, these results suggest that CCM evolution in Euphorbia was likely an adaptive strategy that enabled the occupation of increased arid niche space accompanying Miocene expansion of arid ecosystems. These opportunities evidently facilitated recent, replicated bursts of diversification in Euphorbia. 相似文献
93.
Dr. Ricarda Immel 《Zoomorphology》1955,44(3):163-195
Ohne ZusammenfassungDissertation, Mainz 1954. 相似文献
94.
Stefanie Lehner Marion G?hle Claudia Dierks Ricarda Stelter Jonathan Gerber Ralph Brehm Ottmar Distl 《PloS one》2013,8(12)
Coat color dilution turns black coat color to blue and red color to cream and is a characteristic in many mammalian species. Matings among Netherland Dwarf, Loh, and Lionhead Dwarf rabbits over two generations gave evidence for a monogenic autosomal recessive inheritance of coat colour dilution. Histological analyses showed non-uniformly distributed, large, agglomerating melanin granules in the hair bulbs of coat color diluted rabbits. We sequenced the cDNA of MLPH in two dilute and one black rabbit for polymorphism detection. In both color diluted rabbits, skipping of exons 3 and 4 was present resulting in altered amino acids at p.QGL[37-39]QWA and a premature stop codon at p.K40*. Sequencing of genomic DNA revealed a c.111-5C>A splice acceptor mutation within the polypyrimidine tract of intron 2 within MLPH. This mutation presumably causes skipping of exons 3 and 4. In 14/15 dilute rabbits, the c.111-5C>A mutation was homozygous and in a further dilute rabbit, heterozygous and in combination with a homozygous frame shift mutation within exon 6 (c.585delG). In conclusion, our results demonstrated a colour dilution associated MLPH splice variant causing a strongly truncated protein (p.Q37QfsX4). An involvement of further MLPH-associated mutations needs further investigations. 相似文献
95.
Ricarda A. Busse Andreea Scacioc Javier M. Hernandez Roswitha Krick Milena Stephan Andreas Janshoff Michael Thumm Karin Kühnel 《Autophagy》2013,9(5):770-777
We characterized phosphoinositide binding of the S. cerevisiae PROPPIN Hsv2 qualitatively with density flotation assays and quantitatively through isothermal titration calorimetry (ITC) measurements using liposomes. We discuss the design of these experiments and show with liposome flotation assays that Hsv2 binds with high specificity to both PtdIns3P and PtdIns(3,5)P2. We propose liposome flotation assays as a more accurate alternative to the commonly used PIP strips for the characterization of phosphoinositide-binding specificities of proteins. We further quantitatively characterized PtdIns3P binding of Hsv2 with ITC measurements and determined a dissociation constant of 0.67 µM and a stoichiometry of 2:1 for PtdIns3P binding to Hsv2. PtdIns3P is crucial for the biogenesis of autophagosomes and their precursors. Besides the PROPPINs there are other PtdIns3P binding proteins with a link to autophagy, which includes the FYVE-domain containing proteins ZFYVE1/DFCP1 and WDFY3/ALFY and the PX-domain containing proteins Atg20 and Snx4/Atg24. The methods described could be useful tools for the characterization of these and other phosphoinositide-binding proteins. 相似文献
96.
Five bacterial strains were isolated from polluted soils
capable of degrading 2,2-dichloropropionate. In crude extracts, dehalogenase
activity against haloacetates and longer-chained 2-haloalkanoic acids could
be detected. Results from activity staining indicated that all bacterial
strains expressed a single dehalogenase. In further biochemical
characterization, two types of D,L-specific 2-haloalkanoic acid dehalogenases
were described, which are different from each other not only in molecular
weight and electrophoretic mobility, but also in sensitivity towards thiol
reagents. Dehalogenases of these strains have been shown to be inducible and
are catalyzing halide hydrolysis with inversion of product configuration.
Received: 5 July 1996 / Accepted: 1 August 1996 相似文献
97.
Caruzo MB van Ee BW Cordeiro I Berry PE Riina R 《Molecular phylogenetics and evolution》2011,60(2):193-206
Phylogenetic relationships of Croton section Cleodora (Klotzsch) Baill. were evaluated using the nuclear ribosomal ITS and the chloroplast trnL-F and trnH-psbA regions. Our results show a strongly supported clade containing most previously recognized section Cleodora species, plus some other species morphologically similar to them. Two morphological synapomorphies that support section Cleodora as a clade include pistillate flowers in which the sepals overlap to some degree, and styles that are connate at the base to varying degrees. The evolution of vegetative and floral characters that have previously been relied on for taxonomic decisions within this group are evaluated in light of the phylogenetic hypotheses. Within section Cleodora there are two well-supported clades, which are proposed here as subsections (subsection Sphaerogyni and subsection Spruceani). The resulting phylogenetic hypothesis identifies the closest relatives of the medicinally important and essential oil-rich Croton cajucara Benth. as candidates for future screening in phytochemical and pharmacological studies. 相似文献
98.
99.
Max Seidensticker Ricarda Seidensticker Robert Damm Konrad Mohnike Maciej Pech Bruno Sangro Peter Hass Peter Wust Siegfried Kropf Günther Gademann Jens Ricke 《PloS one》2014,9(11)