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首页 > 医学词汇大全 > Tuberous sclerosis
Tuberous sclerosis

神经

关键词神经 疾病 发育不良性疾病

词汇介绍

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解析

tuberous    英 ['tjuːb(ə)rəs]  美 ['tʊbərəs] 

释    义   adj. 块茎状的;有结节的;隆凸的

同根词   tubercular adj. 结核的;[医] 结节的;瘤状的  

tuberculoid adj. 类似结节的;结核样的  

tuber n. [植] 块茎;隆起

tubercle 结节;[植] 小瘤,[植] 小块茎

tuberosity n.结节;有块茎;块茎状物 

例    句   Subshrubs, or woody stems specialized into tuberous caudex, or absent; anthers withoutappendage; leaves obtuse or acute at apex, sometimes with blunt spines. 亚灌木,或者木的干专业化进块茎状的块根,或无;花药没有附属物;叶钝的或锐尖在先端,有时具钝尖刺。

 

sclerosis    英 [sklɪə'rəʊsɪs; sklə-]  美 [sklə'rosɪs] 

释    义   n. [病理] 硬化,[医] 硬化症;细胞壁硬化 [ 复数 scleroses ]

同根词   sclerosed adj.硬化的;患硬化症的 

sclerotic adj. 硬化的,僵硬的;巩膜的

sclerotic n. [解剖] 巩膜;硬化剂 

sclerotium n. 菌核;硬化体

sclerosed v. 硬化(sclerose的过去式和过去分词)

例    句   All cases had bone thickening and sclerosis of the wall of sinus, and 91.3% cases hadcalcification in the high density soft tissue lesion. 所有病例均有骨增厚和窦壁硬化,91.3 %的病例在高密度软组织病变内还可见钙化。

概述

结节性硬化症是一种以面部血管纤维瘤、癫痫和智力障碍为主要临床表现的复合型发育不良性疾病。属外显不完全的常染色体显性遗传,损害起源于外胚叶或中胚叶,可能与胚胎细胞分化障碍有关。病因和发病机制结节性硬化症由两种不同基因突变引起:位于9q34的TSC1与位于16p13的TSC2,分别编码错构蛋白与马铃薯球蛋白,两种蛋白协同作用,调节细胞生长与抑制。胚胎开始发育时,由于遗传或自发突变造成TSC1或TSC2巾的一个基因缺陷,第—个有丝分裂期出现嵌合体。胚胎在之后的生长发育过程中,含突变的缺陷基因影响另

Iron overload inhibits late stage autophagic flux leading to insulin resistance复制标题

铁过载抑制晚期自噬通量导致胰岛素抵抗

发表时间:2019-10-04

影响因子:8.4

作者: James Won Suk Jahng

期刊:EMBO Rep

Iron overload, a common clinical occurrence, is implicated in the metabolic syndrome although the contributing pathophysiological mechanisms are not fully defined. We show that prolonged iron overload results in an autophagy defect associated with accumulation of dysfunctional autolysosomes and loss of free lysosomes in skeletal muscle. These autophagy defects contribute to impaired insulin-stimulated glucose uptake and insulin signaling. Mechanistically, we show that iron overload leads to a decrease in Akt-mediated repression of tuberous sclerosis complex (TSC2) and Rheb-mediated mTORC1 activation on autolysosomes, thereby inhibiting autophagic-lysosome regeneration. Constitutive activation of mTORC1 or iron withdrawal replenishes lysosomal pools via increased mTORC1-UVRAG signaling, which restores insulin sensitivity. Induction of iron overload via intravenous iron-dextran delivery in mice also results in insulin resistance accompanied by abnormal autophagosome accumulation, lysosomal loss, and decreased mTORC1-UVRAG signaling in muscle. Collectively, our results show that chronic iron overload leads to a profound autophagy defect through mTORC1-UVRAG inhibition and provides new mechanistic insight into metabolic syndrome-associated insulin resistance.

译文

铁超载是一种常见的临床现象,与代谢综合征有关,但其病理生理机制尚未完全阐明。我们发现,长时间的铁超载导致自噬缺陷,与功能失调的自溶体的积累和骨骼肌中游离溶酶体的丢失有关。这些自噬缺陷导致胰岛素刺激的葡萄糖摄取和胰岛素信号传导受损。机制上,我们发现铁超载导致akt介导的结节硬化复合物(tsc2)抑制和rheb介导的mtorc1对自溶体的激活减少,从而抑制自噬性溶酶体再生。mtorc1或缺铁的结构性激活通过增加mtorc1 uvrag信号补充溶酶体池,从而恢复胰岛素敏感性。通过静脉注射右旋糖酐铁诱导小鼠铁超载也会导致胰岛素抵抗,并伴有异常的自噬体积聚、溶酶体丢失和肌肉中mtorc1-uvrag信号传导降低。总之,我们的研究结果表明,慢性铁超载通过抑制mtorc1 uvrag导致严重的自噬缺陷,并为代谢综合征相关的胰岛素抵抗提供了新的机制。