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首页> 外文期刊>Neuropathology: official journal of the Japanese Society of Neuropathology >Overexpression of p53 but not Rb in the cytoplasm of neurons and small vessels in an autopsy of a patient with Cockayne syndrome
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Overexpression of p53 but not Rb in the cytoplasm of neurons and small vessels in an autopsy of a patient with Cockayne syndrome

机译：Cockayne综合征患者尸检中神经元和小血管细胞质中p53的过度表达而不是Rb的表达

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摘要

Cockayne syndrome presents senescence-like changes starting in early infancy; however, the mechanism of premature aging remains unclear. In an autopsy of a 23-year-old woman with Cockayne syndrome, we evaluated the correlation between Cockayne pathology and the expression patterns of the senescence-associated proteins p53 and Rb. Neuropathological findings in this case revealed basal ganglia calcification, tigroid leukodystrophy, bizarre reactive astrocytes, severe cerebellar atrophy with loss of Purkinje cells, and arteriolareuronal calcifications in the hypothalamus. Multiple arteriolar calcifications and sclerotic changes were seen in the central nervous system and kidney, but the endothelium of the aorta and coronary arteries remained intact appropriately for the individual's age without any finding of arteriosclerosis. Overexpression of p53 protein was confirmed in the cytoplasm of neurons in the basal ganglia, thalamus, hypothalamus, hippocampus and cerebellum, of arteriolar endothelial cells of the cerebrum and renal glomerular capillaries, and of cutaneous epithelial cells. The distribution of p53 overexpression was coincident with that of pathological alteration, such as neuronal loss, calcification and atrophy. High expression of p53 was localized in the cytoplasm, not in the nucleus. In contrast to p53, Rb was not expressed in any senescence lesion. In terms of senescence, distinct differences are found among organs in a patient with Cockayne syndrome. This segmental progeria differs from natural aging, and implicates p53 overexpression in the etiology of CS.
机译：Cockayne综合征从婴儿早期就开始出现衰老样变化。然而，过早衰老的机制仍不清楚。在对23名Cockayne综合征妇女的尸检中，我们评估了Cockayne病理与衰老相关蛋白p53和Rb的表达模式之间的相关性。在这种情况下的神经病理学发现显示基底神经节钙化，类脉络膜白细胞营养不良，奇异的反应性星形胶质细胞，严重的小脑萎缩伴浦肯野细胞丢失以及下丘脑小动脉/神经元钙化。在中枢神经系统和肾脏中发现了多个小动脉钙化和硬化改变，但是主动脉内皮和冠状动脉的内皮仍然适合个体的年龄，没有发现任何动脉硬化。在基底神经节，丘脑，下丘脑，海马和小脑，大脑的小动脉内皮细胞和肾小球性毛细血管以及皮肤上皮细胞的神经元细胞质中证实了p53蛋白的过度表达。 p53过表达的分布与神经元丢失，钙化和萎缩等病理改变相一致。 p53的高表达位于细胞质中，而不是细胞核中。与p53相反，Rb在任何衰老病变中均不表达。在衰老方面，患有科卡因综合征的患者的器官之间存在明显差异。这种节段性早衰与自然衰老不同，在CS的病因中涉及p53过表达。

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