排序方式: 共有85条查询结果,搜索用时 15 毫秒
41.
Rosen KM Veereshwarayya V Moussa CE Fu Q Goldberg MS Schlossmacher MG Shen J Querfurth HW 《The Journal of biological chemistry》2006,281(18):12809-12816
Mutations in the ubiquitin ligase-encoding Parkin gene have been implicated in the pathogenesis of autosomal recessive Parkinson disease. Outside of the central nervous system, Parkin is prominently expressed in skeletal muscle. We have found accumulations of Parkin protein in skeletal muscle biopsies taken from patients with inclusion body myositis, a degenerative disorder in which intramyofiber accumulations of the beta-amyloid peptide are pathognomonic. In comparing primary cultures of skeletal muscle derived from parkin knock-out and wild-type mice, we have found the absence of parkin to result in greater sensitivity to mitochondrial stressors rotenone and carbonyl cyanide 3-chlorophenylhydrazone, without any alteration in sensitivity to calcium ionophore or hydrogen peroxide. Utilizing viral expression constructs coding for the Alzheimer disease and inclusion body myositis-linked beta-amyloid precursor protein and for its metabolic byproducts A beta42 and C100, we found that parkin knock-out muscle cells are also more sensitive to the toxic effects of intracellular A beta. We also constructed a lentiviral system to overexpress wild-type Parkin and have shown that boosting the levels of parkin expression in normal skeletal muscle cultures provides substantial protection against both mitochondrial toxins and overexpressed beta-amyloid. Correspondingly, exogenous Parkin significantly lowered A beta levels. These data support the hypothesis that in myocytes parkin has dual properties in the maintenance of skeletal muscle mitochondrial homeostasis and in the regulation of A beta levels. 相似文献
42.
Peter Charbel Issa Samantha R. De Silva Daniel M. Lipinski Mandeep S. Singh Alexandre Mouravlev Qisheng You Alun R. Barnard Mark W. Hankins Matthew J. During Robert E. MacLaren 《PloS one》2013,8(4)
Adeno-associated viral vectors (AAV) have been shown to be safe in the treatment of retinal degenerations in clinical trials. Thus, improving the efficiency of viral gene delivery has become increasingly important to increase the success of clinical trials. In this study, structural domains of different rAAV serotypes isolated from primate brain were combined to create novel hybrid recombinant AAV serotypes, rAAV2/rec2 and rAAV2/rec3. The efficacy of these novel serotypes were assessed in wild type mice and in two models of retinal degeneration (the Abca4−/− mouse which is a model for Stargardt disease and in the Pde6brd1/rd1 mouse) in vivo, in primate tissue ex-vivo, and in the human-derived SH-SY5Y cell line, using an identical AAV2 expression cassette. We show that these novel hybrid serotypes can transduce retinal tissue in mice and primates efficiently, although no more than AAV2/2 and rAAV2/5 serotypes. Transduction efficiency appeared lower in the Abca4−/− mouse compared to wild type with all vectors tested, suggesting an effect of specific retinal diseases on the efficiency of gene delivery. Shuffling of AAV capsid domains may have clinical applications for patients who develop T-cell immune responses following AAV gene therapy, as specific peptide antigen sequences could be substituted using this technique prior to vector re-treatments. 相似文献
43.
Richard Bourbouze Charbel Akiki Isabelle Chardon-Loriaux François Percheron 《Carbohydrate research》1982,106(1):21-30
The evidence for neuraminic acid as a constituent of isolated plant glycoproteins was established by colorimetric and enzymic assay, gas-liquid chromatography-mass spectrometry, and radioactive labeling of glycoproteins containing a terminal sialic acid residue in the carbohydrate chains. 相似文献
44.
Statins Modulate Cyclooxygenase‐2 and Microsomal Prostaglandin E Synthase‐1 in Human Hepatic Myofibroblasts
下载免费PDF全文
![点击此处可从《Journal of cellular biochemistry》网站下载免费的PDF全文](/ch/ext_images/free.gif)
45.
Moussa Cel-H Mitrovic AD Vandenberg RJ Provis T Rae C Bubb WA Balcar VJ 《Neurochemical research》2002,27(1-2):27-35
(2S,1'S,2'R)-2-(Carboxycyclopropyl)glycine (L-CCG III) was a substrate of Na+-dependent glutamate transporters (GluT) in Xenopus laevis oocytes (IC50 13 and 2 M for, respectively, EAAT 1 and EAAT 2) and caused an apparent inhibition of [3H]L-glutamate uptake in mini-slices of guinea pig cerebral cortex (IC50 12 M). In slices (350 M) of guinea pig cerebral cortex, 5 M L-CCG III increased both the flux of label through pyruvate carboxylase and the fractional enrichment of glutamate, GABA, glutamine and lactate, but had no effect on total metabolite pool sizes. At 50 M L-CCG III decreased incorporation of 13C from [3-13C]-pyruvate into glutamate C4, glutamine C4, lactate C3 and alanine C3. The total metabolite pool sizes were also decreased with no change in the fractional enrichment. Furthermore, L-CCG III was accumulated in the tissue, probably via GluT. At lower concentration, L-CCG III would compete with L-glutamate for GluT and the changes probably reflect a compensation for the missing L-glutamate. At 50 M, intracellular L-CCG III could reach > 10 mM and metabolism might be affected directly. 相似文献
46.
Forastieri V Andrade CP Souza AL Silva MS El-Hani CN Moreira LM Mott LR Flores RZ 《Human biology; an international record of research》2002,74(6):861-870
Hall and Kimura (1994) studied the relation between dermatoglyphic asymmetry and male sexual orientation in a sample of 66 homosexual and 182 heterosexual men. They found that more homosexual men possessed a leftward dermatoglyphic asymmetry than did heterosexual men. In this paper, we report a comparative study about the relationship between sexual orientation and dermatoglyphic characteristics, including 60 homosexual men, 76 heterosexual men, and 60 heterosexual women, recruited from the general population, and also from a gay-rights nongovernmental organization, in Salvador, Brazil. Ulnar loops were the most frequent dermatoglyphic pattern in all groups, followed by whorls, arches, and radial loops. A chi-square analysis comparing the frequencies of the patterns in the three groups only showed an excess of ulnar loops in women (p < 0.05) and arches in men (p < 0.01), but did not reveal significant differences between homosexuals and the other groups studied. There was no significant difference between gay and straight men on total ridge count. We found a preponderance of rightward asymmetry in homosexual and heterosexual men, as well as in heterosexual women. Our results do not agree with Hall and Kimura's data indicating that more gay men possessed the minority leftward asymmetry than did straight men. There was no significant difference in leftward asymmetry in the sample studied. The results reported in this paper do not support any relation between dermatoglyphic asymmetry and male sexual orientation, and, thus, any hypothesis concerning a biological intrauterine contribution to adult sexual orientation somehow associated with dermatoglyphic development. 相似文献
47.
Massaad-Massade L Massaad C Legendre F Bas V Chottard J Beaune P Barouki R 《FEBS letters》2000,466(1):49-53
Both cisplatin and the estrogen receptor (ER) are known to bend DNA. The influence of the bending of sequences by the d(GpG)cisPt adduct binding of ER to estrogen response element (ERE)-like sequences was examined. Three ERE-like oligonucleotides with different affinities for ER and which include a GG in the linker sequence were designed in order to form a single central d(GpG)cisPt adduct. Using electrophoretic mobility shift assay and Scatchard analysis, it was shown that the presence of a single d(GpG)cisPt adduct in the linker sequence decreases the ER affinity for DNA. These results do not support a critical role of a DNA bend in the initial recognition of ERE by ER. Then, the platination of DNA outside of the ERE half-sites decreases the interaction of ER with ERE. 相似文献
48.
The entomopathogenic fungi Beauveria bassiana (Balsamo) Vuillemin is known to produce a broad range of secondary metabolites. Beauvericin, a cyclic hexadepsipeptide, is the best known mycotoxin produced by B. bassiana; however, reports discussing the insecticidal activity of beauvericin per se are limited. In this study, we assessed the lethal activity of beauvericin against Tetranychus urticae Koch (Acarina: Tetranychidae). In addition, screening for suitable application of the mycotoxin against T. urticae on greenhouse strawberries is discussed. Beauvericin was able to control successfully T. urticae where concentrations of 10, 100 and 1,000 µg/g recorded mortalities of 84%, 100% and 100%, respectively, against motile stages. Furthermore, beauvericin inhibited egg hatching up to 83.3%, 69.3% and 53.3%, respectively, using the same concentrations under laboratory conditions. Under greenhouse conditions, the efficacy recorded was 52.6%, 85.7%, 72.4% and 72.4% at 1, 3, 7 and 10 days post‐inoculation, respectively. Beauvericin was efficacious under greenhouse conditions since the application increased strawberry yields while showing no phytotoxicity and ecotoxicological risk. Resistance to beauvericin was not detected initially at the unselected strain of T. urticae. Yet, the laboratory selection of populations of T. urticae exposed to beauvericin resulted in relatively resistant T. urticae strain that displayed no cross‐resistance to cyflumetofen and bifenazate. The acaricidal activity of beauvericin documented in this study would increase the efficacy of integrated pest management strategies. 相似文献
49.
Ebermann I Scholl HP Charbel Issa P Becirovic E Lamprecht J Jurklies B Millán JM Aller E Mitter D Bolz H 《Human genetics》2007,121(2):203-211
Usher syndrome is an autosomal recessive condition characterized by sensorineural hearing loss, variable vestibular dysfunction,
and visual impairment due to retinitis pigmentosa (RP). The seven proteins that have been identified for Usher syndrome type
1 (USH1) and type 2 (USH2) may interact in a large protein complex. In order to identify novel USH genes, we followed a candidate
strategy, assuming that mutations in proteins interacting with this “USH network” may cause Usher syndrome as well. The DFNB31 gene encodes whirlin, a PDZ scaffold protein with expression in both hair cell stereocilia and retinal photoreceptor cells.
Whirlin represents an excellent candidate for USH2 because it binds to Usherin (USH2A) and VLGR1b (USH2C). Genotyping of microsatellite
markers specific for the DFNB31 gene locus on chromosome 9q32 was performed in a German USH2 family that had been excluded for all known USH loci. Patients
showed common haplotypes. Sequence analysis of DFNB31 revealed compound heterozygosity for a nonsense mutation, p.Q103X, in exon 1, and a mutation in the splice donor site of
exon 2, c.837+1G>A. DFNB31 mutations appear to be a rare cause of Usher syndrome, since no mutations were identified in an additional 96 USH2 patients.
While mutations in the C-terminal half of whirlin have previously been reported in non-syndromic deafness (DFNB31), both alterations
identified in our USH2 family affect the long protein isoform. We propose that mutations causing Usher syndrome are probably
restricted to exons 1–6 that are specific for the long isoform and probably crucial for retinal function. We describe a novel
genetic subtype for Usher syndrome, which we named USH2D and which is caused by mutations in whirlin. Moreover, this is the first case of USH2 that is allelic to non-syndromic deafness.
Electronic Supplementary Material The online version of this article () contains supplementary material, which is available to authorized users. 相似文献
50.
Jean-Fran?ois Gautier Rapha?l Porcher Charbel Abi Khalil Naima Bellili-Munoz Lila Sabrina Fetita Florence Travert Simeon-Pierre Choukem Jean-Pierre Riveline Samy Hadjadj Etienne Larger Philippe Boudou Bertrand Blondeau Ronan Roussel Pascal Ferré Eric Ravussin Fran?ois Rouzet Michel Marre 《PloS one》2015,10(8)