α- andβ-mannosidoses

Summary Clinical, pathological and biochemical findings in the mannosidoses are described. Family studies showed granulocyte-rich white cell fractions to be the tissue of choice for carrier detection in-mannosidosis. Metabolic labelling studies using [³H] mannose demonstrated accumulation of Man1-4GlcNAc in cultured skin fibroblasts from a patient with this condition. Alternative methods of egress from lysosomes were suggested for this compound by its secretion into culture medium and apparent reduction of storage with time in cultures.-mannosidase deficient goats are not thought to be a true animal model of the human condition, as although they showed a similar enzyme deficiency, the clinical presentation is much more severe and the major storage material (Man1-4GlcNAc1-4GlcNAc) is different.     

Synthesis of four racemic nicotine isotopomers doubly labelled with stable isotope

Four racemic nicotine isotopomers, doubly labelled with stable isotope, were prepared for use in studies of plant metabolism. First, starting from halogeno nicotinates, one deuterium was introduced into the pyridine moiety by reductive dehalogenation with zinc, and choosing deuterated acetic acid as the acidic medium. Pyridine ²H‐labelled nornicotine derivatives were then formed in two reaction steps, a condensation reaction with N‐vinylpyrrolidinone followed by catalytic hydrogenation. The deuteromethylation of these monodeuterated nornicotines was achieved by reduction of the corresponding ethyl carbamates with deuterated lithium aluminium hydride. The four regioisotopomers obtained were at the least 90% labelled on each site, sufficient for the metabolic studies envisaged. Copyright © 2009 John Wiley & Sons, Ltd.     

Feasibility of image registration and intensity-modulated radiotherapy planning with hyperpolarized helium-3 magnetic resonance imaging for non-small-cell lung cancer

PURPOSE: To demonstrate the feasibility of registering hyperpolarized helium-3 magnetic resonance images ((3)He-MRI) to X-ray computed tomography (CT) for functionally weighted intensity-modulated radiotherapy (IMRT) planning. METHODS AND MATERIALS: Six patients with non-small-cell lung cancer underwent (3)He ventilation MRI, which was fused with radiotherapy planning CT using rigid registration. Registration accuracy was assessed using an overlap coefficient, calculated as the proportion of the segmented (3)He-MR volume (V(MRI)) that intersects the segmented CT lung volume expressed as a percentage of V(MRI). For each patient, an IMRT plan that minimized the volume of total lung receiving a dose > or = 20 Gy (V(20)) was compared with a plan that minimized the V(20) to well-ventilated lung defined by the registered (3)He-MRI. RESULTS: The (3)He-MRI and CT were registered with sufficient accuracy to enable functionally guided IMRT planning (median overlap, 89%; range, 72-97%). In comparison with the total lung IMRT plans, IMRT constrained with (3)He-MRI reduced the V(20) not only for the well-ventilated lung (median reduction, 3.1%; range, 0.4-5.1%; p = 0.028) but also for the total lung volume (median reduction, 1.6%; range, 0.2-3.7%; p = 0.028). CONCLUSIONS: Statistically significant improvements to IMRT plans are possible using functional information provided by (3)He-MRI that has been registered to radiotherapy planning CT.     

New complementary perspectives for inpatient physical function assessment: matched clinician-report and patient-report short form measures from the PROMIS adult physical function item bank

Quality of Life Research - To develop two item content-matched, precise, score-level targeted inpatient physical function (PF) short form (SF) measures: one clinician-reported, one...     

一种简捷可靠的小鼠气管插管新方法

目的探索一种安全、高效的小鼠气管插管新方法。方法这种新方法是在小鼠颈前作一小切口,显露气管后,经口腔作气管内插管,费时仅约1 m in。结果经过1300多次实验,无一例失败,成功率达100%。结论这种简捷实用、安全可靠的小鼠气管插管新方法无需特殊设备及体位,一人操作即能完成,可显著提高以小鼠为实验动物模型的成功率。     

B7-1和IL-12基因转染对肝癌细胞免疫原性的影响

目的 观察Ｂ７－１和ＩＬ－１２基因表达对人肝癌细胞免疫原性原影响。方法 分别将Ｂ７－１和ＩＬ－１２基因以逆转录病毒介导转染ＨｅｐＧ２细胞。阳性克隆细胞与健康人外周血淋巴细胞（ＰＢＬ）混合培养后,用流式细胞仪检测ＰＢＬ表面Ⅰ类人白细胞抗原（ＨＬＡ－Ⅰ）分子表达,以ＭＴＴ法检测ＰＢＬ的特异性杀伤活性Ｋ５６２细胞的活性。结果 混合ＨｅｐＧ２／Ｂ７－１ＨｅｐＧ２／ＩＬ－１２细胞组ＰＢＬ表面的ＨＬＡ－Ⅰ分子