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find Keyword "遗传学" 105 results
  • 良性内侧颞叶癫痫的研究进展

    颞叶癫痫(TLE)是指异常过度放电起源于一侧或双侧颞叶结构的癫痫,是最常见的成人局灶性癫痫,也是最常见的药物难治性癫痫。内侧(Mesial)TLE 是 TLE 的主要类型,手术切除致痫灶是有效治疗方法。然而,部分患者的症状则较轻,并且能够在服用或未服用抗癫痫药物治疗的情况下达到无限期的缓解期,被认为是良性(benign)MTLE,此类患者经常有发热发作和癫痫的家族史。目前研究者认为 MTLE 可能与海马硬化和遗传因素有关,文章就 bMTLE 的研究进展进行总结,以提高临床对其认识。

    Release date:2019-01-19 08:54 Export PDF Favorites Scan
  • 表观遗传学调控分子突变在髓系肿瘤中的作用

    基因组研究已经确定在髓系肿瘤包括急性髓系白血病(AML)、骨髓增殖性肿瘤(MPN)和骨髓增生异常综合征(MDS)中,存在多种基因突变,包括DNA甲基转移酶3A、TET甲基胞嘧啶双加氧酶2、异柠檬酸脱氢酶1/2、果蝇zeste基因增强子同源物2和additional sex combs-like 1等,这些表观遗传调控基因突变的发现为髓系肿瘤的研究提供了重要的分子标志和潜在的治疗靶点。该文就AML、MPN和MDS中常见的表观遗传调控基因突变进行综述。

    Release date:2016-10-02 04:54 Export PDF Favorites Scan
  • 早产儿视网膜病变遗传易感性

    除早产以及环境因素以外,遗传因素在早产儿视网膜病变(ROP)的发生发展中亦伴有重要角色。大规模基因扫描和检测确定了一些与ROP相关的基因多态性或基因突变,包括Wnt信号通路相关的Norrie病蛋白、卷曲蛋白4、低密度脂蛋白受体相关蛋白5和四旋蛋白12基因的突变,以及血管内皮生长因子基因、胰岛素样生长因子1基因和其他相关生长因子基因的多态性。尽管这些发现为基因因素在ROP发病机制中的作用提供了很多证据和支持,但仍需要来自不同地区大样本量的比较和分析才能得出有意义的结论。此外,还需要借助生物信息学技术和蛋白组学技术进一步明确ROP的发病机制。ROP可能是累及多个基因的疾病,而非仅仅累及单一基因;每个基因可能贡献较小,但累积到一定量后可能就导致最终的临床表型出现。随着遗传学技术的不断进步,再整合生物信息学和蛋白组学技术,相信将来能够为ROP的治疗提供更好的方案。

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  • 头颜面骨形成不全综合征与视神经萎缩一家系

    Release date:2016-09-02 06:00 Export PDF Favorites Scan
  • 癫痫遗传学分析的临床教学实践探讨

    癫痫是一种由多种原因引起的慢性神经系统疾病,遗传因素在癫痫中的作用日益显著。随着精准医学的快速发展,目前已发现近3 000个基因与癫痫相关,癫痫的遗传学分析在临床诊断和个体化精准治疗中亦日益受到重视,因此提高癫痫专科医生遗传学理论知识、基因变异解读技能以及精准诊疗水平势在必行。本文旨在探讨如何在临床教学实践中进行癫痫遗传学分析教学,我们采取多模式教学方法,包括遗传学理论知识学习、基于案例的学习(case-based learning,CBL)示范教学、一对一教学、病例实操教学、小组讨论教学及临床见习教学,以培养适应精准医学快速发展的高素质创新型医学人才。

    Release date:2024-11-20 10:50 Export PDF Favorites Scan
  • 糖尿病视网膜病变候选基因研究

    糖尿病视网膜病变是一种与血糖水平、病程长短以及遗传与环境因素相互作用所致的复杂疾病,其相关或易感基因研究在近年来非常活跃。利用基因学方法,采用多聚酶链反应等技术,迄今已筛选出了数十种糖尿病视网膜病变的可能相关基因。本文选择介绍了与糖尿病视网膜病变密切相关的几个候选基因的研究进展。 (中华眼底病杂志, 2006, 22: 144-146)

    Release date:2016-09-02 05:51 Export PDF Favorites Scan
  • The relationship of high density lipoprotein cholesterol and cholesterol ester transfer protein TaqIB mutation in non-arteritic anterior ischemic optic neuropathy

    ObjectiveTo investigate the association of high density lipoprotein cholesterol (HDL-C) and cholesterol ester transfer protein (CETP) TaqIB mutation with non-arteritic anterior ischemic optic neuropathy (NA-AION) in the Shaanxi Han ethnic population. MethodsThe study cohort consisted of 45 individuals that had been diagnosed with NA-AION and 45 healthy controls (matched for age, gender). None of the cases or controls had a history of diabetes, serious cardio-cerebral vascular diseases, liver and kidney dysfunction that might influence plasma lipid levels. Plasma HDL-C was detected by enzyme-linked immunosorbent one-step, through the Toshiba TBA-40FR automatic biochemical analyzer. CETP TaqIB gene polymorphism was determined by the polymerase chain reaction-restriction fragment length polymorphism (PCR-RFLP) techniques for analysis. B2B2 genotype was only a fluorescence band with 535 bp; B1B1 genotype was 2 fluorescence bands with 361, 174 bp; B1B2 genotype was 3 fluorescence bands with 535, 361, 174 bp. The relative risk of genotype, HDL-C and disease occurrence was analyzed by logistics regression analysis. ResultsThere have no significant difference between NA-AION patients and controls about plasma total cholesterol level and triglyceride level (t=1.907, 1.877; P > 0.05). The plasma HDL-C levels were significantly lower in NA-AION patients than in controls (t=2.367, P=0.022). Compared with controls, the prevalence of B1B1 genotype and B1 allele was higher (χ2=17.289, P=0.001), the prevalence of B2 allele (χ2=15.648, P=0.000) was lower in NA-AION patients. The lower concentration of HDL-C was risk factor of NA-AION (odds ratio=6.143, 95% confidence interval 1.262-29.895, χ2=27.676;P=0.013). The proportion of B1B1 genotype was significantly higher in NA-AION patients than in controls (odds ratio=2.24, 95% confidence interval 2.427-36.323, χ2=10.526; P=0.001). ConclusionsThe low plasma HDL-C is independent risk factor for NA-AION and is associated with the development of NA-AION in the Shaanxi Han ethnic population. CETP TaqIB mutation is associated with low plasma HDL-C in NA-AION in the Shaanxi Han ethnic population.

    Release date:2016-11-25 01:11 Export PDF Favorites Scan
  • Zinc finger protein 408 in the pathogenesis of familial exudative vitreoretinopathy

    Familial exudative vitreoretinopathy (FEVR) is a rare inherited disorder of retinal angiogenesis, including autosomal dominant, autosomal recessive, or X-linked forms. Zinc finger protein 408 (ZNF408) was recently found to be associated with FEVR. Cell transfection showed that it was a dominant negative regulator of FEVR pathogenesis. Knocking down ZNF408 in zebrafish by antisense morpholino oligonucleotides indicated it involved in retinal blood vessel development. Understanding the protein structure, gene localization, basic functions and the role of ZNF408 in retinal development will contribute to uncover the pathogenesis of FEVR.

    Release date:2016-11-25 01:11 Export PDF Favorites Scan
  • Research progress in epigenetic research on the pathogenesis of retinoblastoma

    Retinoblastoma (RB) is a common intraocular tumor in children, often leading to blindness or disability, and its pathogenesis involves genetic and epigenetic regulation. Epigenetics regulates gene expression through mechanisms such as DNA methylation and histone modification without altering the DNA sequence, and the imbalance of its homeostasis is considered a crucial factor in the development and progression of RB. Therapeutic strategies targeting these abnormal modifications offer new potential treatment avenues for RB. Although current research has highlighted the importance of epigenetics in RB, the specific mechanisms of action, the relationship with genetic bases, and the development of targeted drugs remain largely unknown. Therefore, further in-depth research into the epigenetic mechanisms of RB is of great significance for elucidating its carcinogenic mechanisms, identifying effective therapeutic targets, and developing new drugs.

    Release date:2025-07-17 09:24 Export PDF Favorites Scan
  • 无家族史及系统表现的双眼玻璃体淀粉样变性一例

    Release date:2020-02-18 09:28 Export PDF Favorites Scan
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