Adult-onset type II citrullinemia and idiopathic neonatal hepatitis caused by citrin deficiency: involvement of the aspartate glutamate carrier for urea synthesis and maintenance of the urea cycle

Citrin is a mitochondrial aspartate glutamate carrier primarily expressed in the liver, heart, and kidney. We found that adult-onset type II citrullinemia is caused by mutations in the SLC25A13 gene that encodes for citrin. In this report, we describe the frequency of SLC25A13 mutations, the roles of citrin as a member of the urea cycle and as a member of the malate-aspartate shuttle, the relationship between its functions and symptoms of citrin deficiency, and therapeutic issues.     

Differential effects of Th1, monocyte/macrophage and Th2 cytokine mixtures on early gene expression for glial and neural-related molecules in central nervous system mixed glial cell cultures: neurotrophins, growth factors and structural proteins

Background  

In multiple sclerosis, inflammatory cells are found in both active and chronic lesions, and it is increasingly clear that cytokines are involved directly and indirectly in both formation and inhibition of lesions. We propose that cytokine mixtures typical of Th1 or Th2 lymphocytes, or monocyte/macrophages each induce unique molecular changes in glial cells.     

Upregulation of vascular endothelial growth factor by cobalt chloride-simulated hypoxia is mediated by persistent induction of cyclooxygenase-2 in a metastatic human prostate cancer cell line

Upregulation of vascular endothelial growth factor (VEGF) expression induced by hypoxia is crucial event leading to neovascularization. Cyclooxygenase-2, an inducible enzyme that catalyzes the formation of prostaglandins (PGs) from arachidonic acid, has been demonstrated to be induced by hypoxia and play role in angiogenesis and metastasis. To investigate the potential effect of COX-2 on hypoxia-induced VEGF expression in prostate cancer. We examined the relationship between COX-2 expression and VEGF induction in response to cobalt chloride (CoCl₂)-simulated hypoxia in three human prostate cancer cell lines with differing biological phenotypes. Northern blotting and ELISA revealed that all three tested cell lines constitutively expressed VEGF mRNA, and secreted VEGF protein to different degrees (LNCaP > PC-3 > PC3ML). However, these cell lines differed in the ability to produce VEGF in the presence of CoCl₂-simulated hypoxia. CoCl₂ treatment resulted in 40% and 75% increases in VEGF mRNA, and 50% and 95% in protein secretion by LNCaP and PC-3 cell lines, respectively. In contrast, PC-3ML cell line, a PC-3 subline with highly invasive, metastatic phenotype, exhibits a dramatic upregulation of VEGF, 5.6-fold in mRNA and 6.3-fold in protein secretion after treatment with CoCl₂. The upregulation of VEGF in PC-3ML cells is accompanied by a persistent induction of COX-2 mRNA (6.5-fold) and protein (5-fold). Whereas COX-2 expression is only transiently induced in PC-3 cells and not affected by CoCl₂ in LNCaP cells. Moreover, the increases in VEGF mRNA and protein secretion induced by CoCl₂ in PC-3ML cells were significantly suppressed following exposure to NS398, a selective COX-2 inhibitor. Finally, the effect of COX-2 inhibition on CoCl₂-induced VEGF production was reversed by the treatment with exogenous PGE₂. Our data demonstrate that VEGF induction by cobalt chloride-simulated hypoxia is maintained by a concomitant, persistent induction of COX-2 expression and sustained elevation of PGE₂ synthesis in a human metastatic prostate cancer cell line, and suggest that COX-2 activity, reflected by PGE₂ production, is involved in hypoxia-induced VEGF expression, and thus, modulates prostatic tumor angiogenesis. This revised version was published online in July 2006 with corrections to the Cover Date.     

基于实时三维超声图像的二尖瓣环重建及运动分析

二尖瓣环的非平面特性对二尖瓣返流的超声诊断和二尖瓣环成形术的合理设计具有重要意义.基于心脏的实时三维超声图像,我们研究了一种对二尖瓣环三维重建及运动分析的方法.首先通过人机交互方式提取出二尖瓣环的特征点,并根据位置关系对特征点排序,然后利用非均匀有理B样条曲线建立二尖瓣环三维形态模型,并编程实现二尖瓣环的动态显示和运动分析.通过对20组病例分析,初步证明此方法所建模型较准确反映患者的二尖瓣环的运动,能满足二尖瓣环三维可视化和分析研究的需要.     

半月板的血供及其临床意义

通过动脉灌注,对56侧儿童和成人尸体的半月板血供进行了研究。成人半月板外1/5为血管区,内4/5为无血管区。半月板的撕裂常发生于无血管区或无血管区与血管区的连接处。半月板的退化和磨损位于无血管区。     

Production and characterization of antibody against fusarochromanone

Antibody against fusarochromanone (TDP‐1) was obtained from rabbits after immunization with TDP‐1 conjugated to bovine serum albumin (BSA). An indirect enzyme‐linked immunosorbent assay (ELISA) using TDP‐1‐ovalbumin conjugate as the antigen coated on to the microtiter plate was used for monitoring the antibody liter. For toxin detection, a direct competitive ELISA in which TDP‐1 was conjugated to horseradish peroxidase (HRP) was used. Competitive direct ELISA revealed that the antibody had about 5.6 and 4.5 times greater binding efficiency for monoacetyl fusarochromanone (TDP‐2) and diacetylated TDP‐1 than TDP‐1. The concentration causing 50% inhibition of binding of TDP‐1‐HRP to the antibody by TDP‐1, TDP‐2 and diacetyl‐TDP‐1 were 2.8, 0.5 and 0.62 ng/ml, respectively. For the analysis of fusarochromanones in wheat and barley, the toxins were first extracted from the commodities with 100% methanol. A small aliquot of the extract was dried, acetylated, diluted in buffer and then analyzed directly by ELISA. The overall recovery for fusarochromanone in the wheat and barley samples spiked with TDP‐1 in the concentration range of 20 to 500 ppb were found to be 97% and 103.4% with cv of 15% and 11.2% for barley and wheat, respectively.     

Comparison of hen egg lysozyme immunoassays based on different labels

To check the concentrations of hen egg lysozyme in foodstuffs, added as a bacteriostatic agent, immunoassays based on different labels have been developed. The following detection limits (defined as non‐specific binding increased by three standard deviations) were achieved using antibody labelled with either peroxidase ¹²⁵ I or a biotin‐streptavidin system: 0–8; 0–75 and 0–13 ng/ml, respectively. Only the most sensitive lysozyme immunoassay was likely to be suitable for application to analysis of cheese because matrix interference effects mean that sample extracts need to be diluted prior to assay.     

不同剂量病毒唑治疗肾综合征出血热306例疗效分析

应用不同剂量病毒唑对３０６例肾综合征同血热患者进行治疗对比观察，发现两治疗组疗效无显著性差异，但两治疗组怀对照组相比，越期率高，肾功能恢复快，血小板数恢复早，病程缩短，并发症减少，病死率也有所下降，但无显著性差异。     

国人胃后动脉及其临床意义

本文在查阅了资料后就胃后动脉以下几方面进行综述。1.胃后动脉的起源、出现率、类型;起源于脾动脉者最多,占78％;出现率为62％～88％;绝大部分为一支类型,占75.44～92％。2.外径、长度、毗邻;外径在1.53～3mm之间;长度为2.2～8.1cm;90％的与胃后静脉伴行。3.行径、供血区:在脾动脉上缘发出后,在网膜囊后壁腹膜外向上方走行,经胃膈韧带内最后进入贲门侧的胃底部,并供应该区的血液。     

Inflammatory myofibroblastic tumour (inflammatory pseudotumour) of the breast. Clinicopathological and genetic analysis of a case with evidence for clonality.

Inflammatory myofibroblastic tumours (IMTs) were initially considered to be benign reactive processes, but cases with an unfavourable outcome have been reported. Moreover, clonal genetic alterations have recently been published in some cases, suggesting that IMT may represent a malignant neoplastic entity. This paper reports a case of IMT that developed in the mammary gland, an unusual site. The histological picture was characterized by a proliferation of spindle cells with little cellular atypia and rare mitoses, associated with a polymorphous inflammatory infiltrate. Their immunophenotype, characterized by the expression of vimentin, smooth muscle actin, and cytokeratins, corresponded to that of myofibroblasts. Cytogenetic analysis revealed the clonal nature of the lesion. The modal karyotype was 48, X, ins(2;X)(q34;p21.2p22.2), +7, del(9)(p23), +19. Including the present observation, a 9p deletion has now been found in three cases of IMT. These observations show that IMT may be a clonal neoplasm, even in sites different from deep soft tissues.