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Objective To investigate the clinical, genetic and pathological features of familial chronic progressive external ophthalmoplegia type of mitochondrial myopathy.

目的 探讨家族性慢性进行性眼外肌瘫痪型线粒体肌病的临床、遗传和病理特点。

To study the mitochondrial deletion DNAs of skeletal muscle from patients with chronic progressive external ophthalmoplegia and KearnsSayre syndrome.

目的:检测慢性进行性眼外肌麻痹(chronic progressive external ophthalmoplegia, CPEO)及KearnsSayre综合征患者骨骼肌细胞线粒体DNA的缺失情况。

At mononucleus microspore stage,cytoplasm were thick and organella,such as mitochondria,endoplasmic reticulum were abundant.At two\|nucleus and three\|nucleus stage,there were a lot of starch grains.

结果表明,85 EA、89 AR、TA 与恢复系杂交F1 代小孢子发育表现出明显的可育特征:在单核期,细胞质浓,细胞器丰富,线粒体、内质网等十分发达,二核及三核期淀粉粒数量多。

The otological, audiological and neurological examinations of these patients were conducted, including otoscopy, pure-tone (Madsen 522) and immittance(GSI 33) audiometry. MtDNA A1555G mutation and GJB2 235delC mutation were detected with specific restriction enzyme digestion methods.

对 148 名非综合征型感音神经性耳聋患者分别进行线粒体 DNA 12SrRNA基因A1555G 点突变和 GJB2 基因235delC突变的限制性内切酶分析。

Methods Pancreatic cancer cell panc-1 was treated with oxamate for 48 h,the mitochondrial membrane potential was measured by flow cytometry(Rodamin-123),and the activity of intracellular lactate dehydrogenase was studied by enzymohistochemistry.

方法体外培养人胰腺癌细胞panc-1,分别加入不同浓度的草氨酸盐培养48 h后,行酶组织化学染色及罗丹明123染色,观察细胞糖酵解关键酶乳酸脱氢酶活性及线粒体膜电位。

It is proved by ultrastructural observations that few plastids and abundednt mitochondria were ever presenting in the generative cell of Pisum sativum from its formation to maturation.

在豌豆中证明了生殖细胞自形成直至成熟花粉时,始终存在少量质体和较多的线粒体。

IL possesses the protective effects on procerebrum ischemia/reperfusion injury in mice and its mechanism may be related to anti-oxidant action and improvement of the energy metabolism.

IL对实验性前脑I/R损伤的保护作用机制可能与减少脑组织和脑细胞线粒体中过氧化物和氧自由基含量,增高SOD活性及改善ATPase活性有关。

After the first division of the zygote, organelles from male and female gametes appear to be equally incorperated into each cell of the two-celled proembryo.

中央细胞极核周围的细胞质中有各种细胞器,包括质体、线粒体、粗面内质网和高尔基体。

Observed with transmission electronic microscope,ES-like cell have obvious nucleolus and a little heterochromatin,and many ribosomes in the cytoplasm with much endoplasmic reticulum and mitochondria. A part of cells appeared alveoli on the nuclear envelope,and chromatin was pycnosis and the .

在透射电镜下细胞核仁明显、核异染色质少;细胞质中核糖体较多,内质网、线粒体发达,部分细胞的细胞核膜上出现小泡,染色质边集,染色质形成凋亡小体。

Fig.1 SHEE cultured on coverslide, the living cells were growing in single layer with rich cytoplasm, the nuclei were uniform in size with a nucleolus ph ×400 Fig.2 SHEE had a nucleus with ellipse shape, large nucleolus and the cytoplasm contained mitochondria and tonofibrilEM ×10 000 Fig.3 SHEE was spherical in shape, with pseudopods attached on petri dish and abundant villi on cell surface SEM ×5 000 Fig.4 Same as in Fig.3, cell attached on petri dish, appeared stellate or polygonal in shape, with abundant pseudopods and cytoplasmic processes. Protrusive nuclear region in central part of the cell had more micro-villi SEM ×5 000 Fig.5 Chromosomes of SHEE cells belonged to diploidy type Giemsa ×1 000 Fig.6 The SHEE cells of stained in dark brown by Ki67 immunohistochemistry were the proliferative cells Immunohistochemistry ×400 Fig.7 In SHEE cell culture, the nucleus stained red or pink by PI was dead cell, the green nucleus was living cell Fluorescent ×400 Fig.8 The cell labeled by TdT was apoptotic cell in which the chromatin of nucleus condensed in block, a pyknotic nucleus in the upper right conner was seen TdT labeled ×400

图1 SHEE培养在盖坡片上,活细胞单层生长,胞浆较丰富,细胞核大小一致,有核仁×400 图2 SHEE培养细胞细胞核椭圆形,核仁较大,胞浆有较丰富的线粒体和张力原纤维EM ×10 000 图3 SHEE细胞呈球状,有伪足贴壁,表面有密集微绒毛SEM ×5 000 图4 同上细胞贴壁,呈星状或多角形,有丰富伪足和胞浆突,核区隆起有较多微绒毛SEM ×5 000 图5 SHEE细胞染色体仍属二倍体Giemsa染色×1 000 图6 SHEE细胞Ki67免疫组织化学染棕黄色为增殖细胞×400 图7 SHEE培养细胞出现死细胞,胞核和胞浆PI染色呈红色或淡红色,蓝色细胞核为活细胞荧光显微镜×400 图8 细胞TdT标记阳性为凋亡细胞,染色质凝集呈块状,右上角有一固缩细胞核TdT标记×400

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