加拿大pc

龙虎斗手机游戏bg真人试玩韦德投注vr彩票平台官方网址bbinapp官网体育投注bg娱乐app平台下载ag旗舰厅手机版app亚博vip12极速体育官网bg视讯官方网站伟德app 一彩票app手机版下载188体育首页亚博软件怎么下bet必威登录im电竞im体育下载亚洲十大信誉平台千亿体育官网yabo官网平台188体育平台在线1im电竞抢庄牛牛可提现下载
论文相关信息

 20 december,2018|Supp Info:

The lysine catabolite saccharopine impairs development by disrupting mitochondrial homeostasis

Junxiang Zhou1,2,3, Xin Wang2 , Min Wang1, Yuwei Chang4, Fengxia Zhang4, Zhaonan Ban3,4, Ruofeng Tang1,3, Qiwen Gan1,3, Shaohuan Wu3,4,5, Ye Guo1, Qian Zhang1,3, Fengyang Wang1,3, Liyuan Zhao1,3, Yudong Jing1 , Wenfeng Qian4,5, Guodong Wang4, Weixiang Guo1 , and Chonglin Yang1,2

 

Abstract

Amino acid catabolism is frequently executed in mitochondria; however, it is largely unknown how aberrant amino acid metabolism affects mitochondria. Here we report the requirement for mitochondrial saccharopine degradation in mitochondrial homeostasis and animal development. In Caenorhbditis elegans, mutations in the saccharopine dehydrogenase (SDH) domain of the bi-functional enzyme α-aminoadipic semialdehyde synthase AASS-1 greatly elevate the lysine catabolic intermediate saccharopine, which causes mitochondrial damage by disrupting mitochondrial dynamics, leading to reduced adult animal growth. In mice, failure of mitochondrial saccharopine oxidation causes lethal mitochondrial damage in the liver, leading to postnatal developmental retardation and death. Importantly, genetic inactivation of genes that raise the mitochondrial saccharopine precursors lysine and α-ketoglutarate strongly suppresses SDH mutation-induced saccharopine accumulation and mitochondrial abnormalities in C. elegans. Thus, adequate saccharopine catabolism is essential for mitochondrial homeostasis. Our study provides mechanistic and therapeutic insights for understanding and treating hyperlysinemia II (saccharopinuria), an aminoacidopathy with severe developmental defects.





bet9九州体育10年信誉bbin游戏平台百家乐官网官方网站a8体育平台澳门盘口足球即时赔率电竞博彩app哪个好电竞比赛押注appag官方电子游戏ag真人在线泛亚电竞在线飞艇彩票正规平台金沙澳门登录网站 吃鸡竞猜足球波胆预测西甲买球bg真人appAOA体育官网推荐**亚搏体育登录大阳城娱乐手机版亚伯体育泛亚电竞英雄联盟竞猜bob体彩官网球会体育平台澳门js网站老金沙网址 bg真人比分直播188足球dt电子娱乐游戏葡京登陆金沙官网登陆足球直播免费观看球会体育平台极速快三广东贵宾会网址大发国际EBET真人网站三升体育平台