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Background: Retinal pigment epithelium (RPE)lesions are predictive congenital phenotypic markersfor familial adenomatous polyposis (FAP). Thisprospective screening study aims at assessing theincidence and significance of these lesions in FAPpatients and their family members.Methods: Sixty-two members from three familiesincluding five patients with the diagnosis of FAP havebeen ophthalmologically surveyed. All RPE lesions weredocumented with fundus photography and fluoresceinangiography was performed in 13 subjects.Sigmoidoscopy and/or radiological examination wereperformed annually in 9 family members with typicalRPE lesions during 4 years to allow early diagnosis ofFAP.Results: Typical RPE lesions were present infive FAP patients and 15 family members.Telangiectatic dilatations in the retinal peripherywith small dot-like hemorrhages were detected in 6subjects from 3 families These lesions wereparticularly evident on fluorescein angiography.Annual colon analysis showed polyps in 3 out of 9subjects who were positive for RPE lesions.Conclusion: RPE lesions are valuable as aclinical marker in predicting FAP. The co-existingperipheral vascular alterations which have not beenreported before, are probably related to FAP.  相似文献   
3.
Matrix metalloproteinases (MMPs) and the tissue inhibitors of metalloproteinases (TIMPs) play an essential role in both normal and pathological extracellular matrix degradation, and a TIMP has been associated with at least one type of retinal degeneration. We have studied expression of MMP-2 and TIMP-1 by zymography, immunocytochemistry, and immunoblotting in the retinal pigment epithelium (RPE) from normal, aged and diseased retinas. MMPs and TIMPs were found in the rat RPE, interphotoreceptor matrix (IPM), and in media conditioned by human and rat RPE in culture. In other polarized cells, MMPs and TIMP-2 are secreted vectorially towards the basal lamina. In the RPE, however, MMP-2 and TIMP-1 were secreted preferentially from the apical surface, the surface bordering the IPM. These findings provide new evidence that MMPs and TIMPs could play a role in the turnover of IPM components.Cell homogenates and conditioned media from RPE isolated from mutant Royal College of Surgeons (RCS) rats with inherited retinal dystrophy had similar amounts of MMP-2 and TIMP-1 as those from congenic control rats. The secretion of MMP-2 and TIMP-1 from RPE cell cultures isolated from young and aged human donors varied widely. However, with increasing cell passage number, secretion of MMPs and TIMPs from human RPE increased dramatically. Also, growing human RPE on bovine corneal endothelial cell-generated extracellular matrix instead of plastic reduced the secretion of both MMPs and TIMPs. These data suggest that the integrity of Bruch's membrane may serve to regulate RPE functions in MMP and TIMP secretion and that extracellular matrices contain signals that regulate MMP and TIMP synthesis and/or secretion by the RPE.  相似文献   
4.
海藻、甘草及其相伍用对小鼠肝药酶的影响   总被引:5,自引:0,他引:5  
目的:研究海藻、甘草单煎液及其不同比例合煎,单煎后混合液以及单体A、B;C、D、E对小鼠肝药酶的影响。方法:取小鼠随机分组,分别ig给药,qd,连续4d后禁食20h,处死取肝脏,称重后制成匀浆,紫外分光光度计于450nm,490nm波长下测其光密度值,计算肝匀浆中细胞色素P-450的含量。结果:甘草、甘草与海藻合煎液及单体A,B,C,D,E均能显著提高小鼠肝匀浆中细胞色素P-450的含量,而海藻,甘草与海藻单煎后混合液未能提高小鼠肝心浆中的细胞色素P-450的含量。结论:甘草,甘草与海藻合煎液及单体,A,B,C,D,E均能显著提高小鼠肝匀浆中细胞色素P-450的含量,对肝药酶有诱导作用。  相似文献   
5.
Retinol-binding protein (RBP) is the transport protein that carries retinol in the circulation from the liver to its target tissues. The existence of a cell-surface receptor on the target cells, which mediates the uptake of retinol from RBP, has been known since 1975. Recently, it was identified as an integral transmem-brane protein named STRA6 that is inducible by retinoic acid in certain cancer cells. The receptor was found to be highly specific for RBP, with high affinity, and to be localized in all tissues known to require retinol for their function, particularly the pigment epithelium of the eye.  相似文献   
6.
以制备晶形、粒度、轴比等满足高性能磁粉要求的铁黄为目标,研究了碱法超细α-FeOOH合成的工艺配方和工艺条件对粒子性能的影响,包括碱比、气量、转速、Fe(OH)_2结晶条件、熟化时间等工艺参数的影响规律。重复稳定地制备出三类超微粒α-FeOOH粒子,其粒度分别为0.18、0.28、0.39μm,分布均匀,无枝叉。  相似文献   
7.
Stephen Girard trader, banker, millionaire, and patriot lived 81 full and exciting years. Apparently born with a blind or amblyopic right eye, he emigrated from Bordeaux. France, via Santa Domingo to the United States, and finally settled in Philadelphia, where he developed a successful maritime trade. Ultimately, Girard became the first multimillionaire in the United States. Extremely generous, he often walked from his home in the center of Philadelphia to his farm in what is now South Philadelphia distributing shoes to needy children. Probably Girard’s most heroic gestures were his gallant fight against the yellow fever epidemic in 1793 and his loan to the U.S. government during the War of 1812, which allowed the bankrupt country to continue the conflict and ultimately to win the war. Presented, in part, at the tenth annual meeting of the David G. Cogan Ophthalmic History Society, the College of Physicians of Philadelphia, March 8 and 9, 1997.  相似文献   
8.
The development of aberrant pigmentation represents an unwelcome complication to an otherwise successful split skin graft resulting in a loss of colour match and, so it follows, of cosmesis. We present two cases where lasers have been successful in the treatment of this problem.  相似文献   
9.
荔枝壳红色素的提取及稳定性观察   总被引:4,自引:0,他引:4  
以荔枝壳为原料,在酸性条件下浸提荔枝壳红色素,并对其稳定性进行了研究,结果表明,该色素在酸性条件下对光、热、常用食品添加剂均具有较好的稳定性。  相似文献   
10.
目的研究微囊化后的猪视网膜色素上皮细胞(retinal pigment epithelial,RPE)对帕金森病大鼠模型的移植疗效。方法原代培养RPE 并传代,高效液相色谱法测定培养液上清中多巴胺(dopamine, DA)和高香草酸(homovanillic acid, HVA)的含量,ELISA法检测脑源性神经营养因子(brain-derived neurotrophic factor, BDNF)和胶质细胞源性神经营养因子(glial-derived neurotrophic factor, GDNF)的含量。用高压静电成囊装置制备海藻酸钠-多聚赖氨酸-海藻酸钠微囊化细胞。6-羟基多巴胺(6-hydroxydopamine, 6-OHDA)毁损内侧前脑束 (medial fore-brain bundle,MFB)建立 SD 大鼠帕金森病模型。立体定向移植 RPE+ 微囊,检验旋转实验、免疫组化和脑内生化的变化。结果 RPE 培养上清液中DA、HVA、BDNF、GDNF 的含量稳定,微囊化后细胞长期存活,活性没有明显变化。6-OHDA毁损MFB建立大鼠帕金森病模型的成模率为83%。移植微囊化的RPE后有效率为33%。结论猪 RPE 体外培养生长旺盛,持续分泌 DA、BDNF 和 GNDF,微囊化不影响其分泌功能。RPE 移植对帕金森病大鼠有一定的治疗作用。  相似文献   
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