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首页 > 医学词汇大全 > Amyotrophic lateral sclerosis
Amyotrophic lateral sclerosis

儿科

关键词儿科 疾病 瘫痪性疾病

词汇介绍

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解析

amyotrophic 英 /eimaiə'trɔfik/  美 /eimaiə'trɔfik/

释    义   adj. 肌萎缩的

例    句   For around a decade, scientists have been trying to regrow nerve cells lost in neurodegenerativediseases such as Parkinson's, Alzheimer's and amyotrophic lateral sclerosis (ALS) from stem cells.大约十年间,科学家们一直尝试利用干细胞使神经退变性疾病中的神经细胞再生,例如帕金森病,阿尔茨默病和肌萎缩侧索硬化。

 

lateral 英 /ˈlætərəl/  美 /ˈlætərəl/

释    义   n. 侧部;[语] 边音     

adj. 侧面的,横向的     

vt. 横向传球

例    句   At this point you must alternate lateral and radial truing . Be patient. 在这点上你必须交替的从侧面和纵向进行调整。

 

sclerosis 英 /skləˈrəʊsɪs/ 美 /skləˈroʊsɪs/

释    义   n. [病理] 硬化,[医] 硬化症;细胞壁硬化

例    句   Tuberous sclerosis can lead to the formation of fibroids or tumors in the pancreas, bone, and liver.结节性硬化症可以导致患者的胰腺、骨骼和肝脏发生纤维瘤或其他肿瘤。

概述

肌萎缩侧索硬化(ALS)是一种进展性瘫痪性疾病,以脑和脊髓运动神经元的退行性变为特点。它以局部无力隐匿起病,但无情地扩展累及大多数肌肉,包括膈肌。通常在3到5年内出现呼吸肌麻痹而死亡。流行病学特点在欧洲和美国,每10万人每年有1-2个新发ALS患者,每10万人的总患病人数约为3-5。这些统计数据在全球相对一致,尽管在少数地区ALS更常见。ALS的发病率和患病率随年龄增长。在美国和欧洲,终生累积患ALS的风险约为1/400,单在美国,预计现在活着的人将有80万死于ALS。约10%的ALS患者是

C9orf72 expansion within astrocytes reduces metabolic flexibility in amyotrophic lateral sclerosis复制标题

星形胶质细胞内的C9orf72扩张降低肌萎缩侧索硬化的代谢灵活性

发表时间:2019-10-24

影响因子:11.8

作者: Allen Scott P

期刊:Brain

It is important to understand how the disease process affects the metabolic pathways in amyotrophic lateral sclerosis and whether these pathways can be manipulated to ameliorate disease progression. To analyse the basis of the metabolic defect in amyotrophic lateral sclerosis we used a phenotypic metabolic profiling approach. Using fibroblasts and reprogrammed induced astrocytes from C9orf72 and sporadic amyotrophic lateral sclerosis cases we measured the production rate of reduced nicotinamide adenine dinucleotides (NADH) from 91 potential energy substrates simultaneously. Our screening approach identified that C9orf72 and sporadic amyotrophic lateral sclerosis induced astrocytes have distinct metabolic profiles compared to controls and displayed a loss of metabolic flexibility that was not observed in fibroblast models. This loss of metabolic flexibility, involving defects in adenosine, fructose and glycogen metabolism, as well as disruptions in the membrane transport of mitochondrial specific energy substrates, contributed to increased starvation induced toxicity in C9orf72 induced astrocytes.

译文

了解疾病过程如何影响肌萎缩侧索硬化症的代谢途径,以及这些途径是否可以被操纵以改善疾病进展是很重要的。为了分析肌萎缩侧索硬化症代谢缺陷的基础,我们采用表型代谢谱分析方法。利用C9ORF72和散发性肌萎缩性侧索硬化患者的成纤维细胞和重编程诱导的星形胶质细胞,同时测定了91种势能底物还原性烟酰胺腺嘌呤二核苷酸(NADH)的产生率。我们的筛选方法表明,C9OFF72和散发性肌萎缩侧索硬化引起的星形胶质细胞具有不同的代谢概况与对照相比,并显示了损失的代谢灵活性,没有观察到成纤维细胞模型。代谢灵活性的丧失,包括腺苷、果糖和糖原代谢的缺陷,以及线粒体特异性能量底物的膜转运的破坏,导致C9OFF72诱导的星形胶质细胞饥饿诱导的毒性增加。