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排序方式: 共有104条查询结果,搜索用时 15 毫秒
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Willeke MC van Roon-Mom Barry A Pepers Peter AC 't Hoen Carola ACM Verwijmeren Johan T den Dunnen Josephine C Dorsman GertJan B van Ommen 《BMC molecular biology》2008,9(1):84
Background
Huntington's disease is a progressive autosomal dominant neurodegenerative disorder that is caused by a CAG repeat expansion in the HD or Huntington's disease gene. Although micro array studies on patient and animal tissue provide valuable information, the primary effect of mutant huntingtin will inevitably be masked by secondary processes in advanced stages of the disease. Thus, cell models are instrumental to study early, direct effects of mutant huntingtin. mRNA changes were studied in an inducible PC12 model of Huntington's disease, before and after aggregates became visible, to identify groups of genes that could play a role in the early pathology of Huntington's disease. 相似文献94.
The sugar binding activity of MR60, a mannose-specific shuttling lectin, requires a dimeric state 总被引:1,自引:0,他引:1
MR60 is an intracellular membrane protein which has been shown to act as a
mannoside specific lectin and to be identical to ERGIC-53, a protein
characteristic of the endoplasmic reticulum-Golgi apparatus- intermediate
compartment, acting as a shuttle. According to its primary sequence, this
MR60/ERGIC-53 protein contains a luminal domain including the carbohydrate
recognition domain, a stem, a transmembrane segment and a cytosolic domain.
The endogenous MR60/ERGIC-53 protein is spontaneously oligomeric, (dimers
and hexamers). In this paper, we study the relationship between the
oligomerization state and the sugar binding capacity by using recombinant
proteins. The expression of the recombinant proteins was evidenced by
immunocytochemistry and by immunoprecipitation followed by SDS-PAGE
analysis. The full size recombinant protein binds mannosides and is
oligomeric, up to the hexameric form. Two truncated proteins lacking the
transmembrane and the cytosolic domains were prepared and characterized. A
long one, containing the cysteine 466 close to the C-terminal end of the
recombinant protein but lacking the cysteine 475, close to the C- terminal
end of the native protein, does bind mannosides and forms dimers but no
higher oligomeric forms. A shorter one, lacking both the cysteines 466 and
475, does not bind mannosides and does not form dimers or higher polymers.
The two cysteines in the carbohydrate recognition domain (C190 and C230)
are not involved in the stabilization of oligomers. In conclusion, this
study shows that the luminal moiety of MR60/ERGIC-53 contains a device
allowing both its oligomeric pattern and its sugar binding capability.
相似文献
95.
C Melzer AC Borges F Knebel WS Richter W Combs G Baumann H Theres 《Cardiovascular ultrasound》2004,2(1):1-7
Background
Power Doppler (PD) has improved diagnostic capabilities of vascular sonography, mainly because it is independent from the angle of insonation. We evaluated this technique in a prospective comparison with conventional imaging, consisting in Duplex and Color Doppler, for the evaluation of Renal Artery (RA) stenosis.Methods
Sensitivity, specificity and predictive values of PD and conventional imaging were assessed in a blinded fashion on eighteen patients, 9 with angiographic evidence of unilateral RA stenosis (hypertensive patients) and 9 with angiographically normal arteries (control group). PD images were interpreted with an angiography-like criteria.Results
In the control group both techniques allowed correct visualization of 16 out of the 18 normal arteries (93% specificity). Only in five hypertensive patients RA stenosis was correctly identified with conventional technique (56% sensitivity and 86% negative predictive value); PD was successful in all hypertensive patients (100% sensitivity and negative predictive value), since the operators could obtain in each case of RA stenosis a sharp color signal of the whole vessel with a clear "minus" at the point of narrowing of the lumen. All results were statistically significant (p < 0.01).Conclusions
This study demonstrates that PD is superior to conventional imaging, in terms of sensitivity and specificity, for the diagnosis of RA stenosis, because it allows a clear visualization of the whole stenotic vascular lumen. Especially if it is used in concert with the other sonographic techniques, PD can enable a more accurate imaging of renovascular disease with results that seem comparable to selective angiography. 相似文献96.
Yasuyuki Kobayashi Jon T Giles Masaharu Hirano Isamu Yokoe Yasuo Nakajima Joan M Bathon Joao AC Lima Hitomi Kobayashi 《Arthritis research & therapy》2010,12(5):R171
Introduction
Rheumatoid arthritis (RA) is a multi-organ inflammatory disorder associated with high cardiovascular morbidity and mortality. We sought to assess cardiac involvement using a comprehensive cardiac magnetic resonance imaging (cMRI) approach and to determine its association with disease characteristics in RA patients without symptomatic cardiac disease. 相似文献97.
Timm Konold Yoon Hee Lee Michael J Stack Claire Horrocks Robert B Green Melanie Chaplin Marion M Simmons Steve AC Hawkins Richard Lockey John Spiropoulos John W Wilesmith Gerald AH Wells 《BMC veterinary research》2006,2(1):1-20
Background
Given the theoretical proposal that bovine spongiform encephalopathy (BSE) could have originated from sheep scrapie, this study investigated the pathogenicity for cattle, by intracerebral (i.c.) inoculation, of two pools of scrapie agents sourced in Great Britain before and during the BSE epidemic. Two groups of ten cattle were each inoculated with pools of brain material from sheep scrapie cases collected prior to 1975 and after 1990. Control groups comprised five cattle inoculated with sheep brain free from scrapie, five cattle inoculated with saline, and for comparison with BSE, naturally infected cattle and cattle i.c. inoculated with BSE brainstem homogenate from a parallel study. Phenotypic characterisation of the disease forms transmitted to cattle was conducted by morphological, immunohistochemical, biochemical and biological methods.Results
Disease occurred in 16 cattle, nine inoculated with the pre-1975 inoculum and seven inoculated with the post-1990 inoculum, with four cattle still alive at 83 months post challenge (as at June 2006). The different inocula produced predominantly two different disease phenotypes as determined by histopathological, immunohistochemical and Western immunoblotting methods and biological characterisation on transmission to mice, neither of which was identical to BSE. Whilst the disease presentation was uniform in all scrapie-affected cattle of the pre-1975 group, the post-1990 inoculum produced a more variable disease, with two animals sharing immunohistochemical and molecular profile characteristics with animals in the pre-1975 group.Conclusion
The study has demonstrated that cattle inoculated with different pooled scrapie sources can develop different prion disease phenotypes, which were not consistent with the phenotype of BSE of cattle and whose isolates did not have the strain typing characteristics of the BSE agent on transmission to mice. 相似文献98.
Copper sulfate can cause different pathologies in different organ systems during development. We determined the effects of toxic levels of copper sulfate on brain development in term Hubbard broiler chicks using stereological and biochemical analyses. Hubbard broiler chicken eggs were divided into three groups: controls with no treatment, sham-treated animals and an experimental group. On day 1, 0.1 ml saline was injected into the air chambers of the sham and experimental groups. The experimental group received also 50 μg copper sulfate. At term (day 21), all chick brains were removed and their volumes were determined using the Cavalieri volume estimation. Parallel biochemical analyses were carried out for glutathione and malondialdehyde levels in the brain tissues as indicators of oxidative damage. With copper treatment, the mean brain volume (8079 μm3) was significantly decreased compared to both the control (10075 μm3) and sham (9547 μm3) groups. Copper treatment (143.8 nmol/g tissue) showed significantly decreased malondialdehyde levels compared to the control (293.6 nmol/g tissue) and sham groups (268.8 nmol/g tissue). Copper treatment (404.5 nmol/g tissue) showed significantly increased malondialdehyde levels compared to the control (158.6 nmol/g tissue) and sham (142.8 nmol/g tissue) groups. The morphological and biochemical parameters we measured demonstrated that in term Hubbard broiler chicks, toxic levels of copper sulfate cause developmental and oxidative brain damage. 相似文献
99.