MITOCHONDRIAL

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相关机构：北京大学中国科学院中国人民解放军总医院中国科学院遗传与发育生物学研究所更多>>
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Compound heterozygous mutation of AFG3L2 causes autosomal recessive spinocerebellar ataxia through mitochondrial impairment and MICU1 mediated Ca2+overload
《Science China(Life Sciences)》2025年第2期484-501,共18页Hongyu Li Qingwen Ma Yan Xue Linlin Cai Liwen Bao Lei Hong Yitao Zeng Shu-Zhen Huang Richard H.Finnell Fanyi Zeng 
supported by the National Key Research and Development Program of China(2019YFA0801402);the National Natural Science Foundation of China(82271890);the Shanghai Key Clinical Specialty Project(shslczdzk05705);the Shanghai Top Priority Key Discipline Project(2017ZZ02019);Innovative Research Team of High-Level Local Universities in Shanghai(SHSMU-ZDCX20212200);the Macao Science and Technology Development Fund(FDCT)(0092/2022/A2,003/2022/ALC)。
Autosomal recessive spinocerebellar ataxias(SCARs)are one of the most common neurodegenerative diseases characterized by progressive ataxia.Although SCARs are known to be caused by mutations in multiple genes,there ar...
关键词：AFG3L2 gene autosomal recessive spinocerebellar ataxia mitochondrial impairment Ca2+overload 
MSC-mediated mitochondrial transfer restores mitochondrial DNA and function in neural progenitor cells of Leber’s hereditary optic neuropathy
《Science China(Life Sciences)》2024年第11期2511-2519,共9页Rui Wang Feixiang Bao Manjiao Lu Xiaoyun Jia Jiahui Xiao Yi Wu Qingjiong Zhang Xingguo Liu 
financially supported by the National Key Research and Development Program of China(2022YFE0210100,2023YFE0210100,2022YFA1103800,2019YFA0904500);the National Natural Science Foundation projects of China(32025010,92157202,32241002,92254301,92357302,32261160376,31970709,32070729,32100619,32170747,32322022,32370782,32371007,32300608,32300620);NSFC/RGC Joint Grant Scheme 2022/2023(N_CUHK 428/22);the Strategic Priority Research Program of the Chinese Academy of Sciences(XDB0480000);the Key Research Program,CAS(ZDBS-ZRKJZ-TLC003);International Cooperation Program,CAS(154144KYSB20200006);CAS Project for Young Scientists in Basic Research(YSBR-075);Guangdong Province Science and Technology Program(2023B0303000023,2023B1111050005,2023A1515030231,2022A1515110493,2023B1212060050,2021A1515012513,2021B1515020096,2022A1515012616,2022A1515110951,2023B1212120009,2024A1515010782,2024B1515040020,2024A1515030120);Guangzhou Science and Technology Program(202102021037,202102020827,202102080066,202206060002,2023A04J0414);Health@InnoHK funding support from the Innovation Technology Commission of the Hong Kong SAR,Basic Research Project of Guangzhou Institutes of Biomedicine and Health,Chinese Academy of Sciences;CAS Youth Innovation Promotion Association(to Y.W and K.C).
Leber’s hereditary optic neuropathy(LHON)is a debilitating mitochondrial disease associated with mutations in mitochondrial DNA(mtDNA).Unfortunately,the available treatment options for LHON patients are limited due t...
关键词：MITOCHONDRIA mitochondrial DNA mitochondrial diseases induced pluripotent stem cells stem cells METABOLISM energy mesenchymal stem cells 
Short-form OPA1 is a molecular chaperone in mitochondrial intermembrane space被引量：1
《Science China(Life Sciences)》2022年第2期227-235,共9页Deyang Yao Yukun Li Sheng Zeng Zhifan Li Zahir Shah Bigui Song Jinglei Liu Yi Wu Liang Yang Qi Long Wenqian Wang Zhijuan Hu Haite Tang Xingguo Liu 
the National Key Re-search and Development Program of China(2017YFA0106300);the Stra-tegic Priority Research Program of the Chinese Academy of Sciences(XDA16030505);the National Key Research and Development Program of China(2017YFA0102900,2019YFA09004500,2017YFC1001602,2016YFA0100300,2018YFA0107100);the National Natural Science Foundation of China(32025010,31801168,31900614,31970709,81901275,32070729);the Key Research Program of Frontier Sciences,CAS(QYZDB-SSW-SMC001);International Cooperation Program,CAS(154144KYSB20200006);Guangdong Province Science and Technol-ogy Program(2020B1212060052,2018A030313825,2018GZR110103002,2020A1515011200,2020A1515010919,2020A1515011410,2021A1515012513);Guangzhou Science and Technology Program(201807010067,202002030277,202102021250,202102020827,202102080066);CAS Youth Innovation Promotion Association.
Mitochondria,double-membrane organelles,are known to participate in a variety of metabolic and signal transduction pathways.The intermembrane space(IMS)of mitochondria is proposed to subject to multiple damages emanat...
关键词：MITOCHONDRIA OPA1 CHAPERONE mitochondrial homeostasis heat shock 
Mitochondria determine the sequential propagation of the calcium macrodomains revealed by the super-resolution calcium lantern imaging
《Science China(Life Sciences)》2020年第10期1543-1551,共9页Yulin Zhang Jianyong Wang Shijia Xing Liuju Li Shiqun Zhao Wenzhen Zhu Kuo Liang Yanmei Liu Liangyi Chen 
Supported by the grants from the National Science and Technology Major Project Program(2016YFA0500400);the National Natural Science Foundation of China(81925022,91854112,31327901,31521062,31570839,91750203);and Beijing Natural Science Foundation(L172003,7182063).
Despite the wide application of super-resolution(SR)microscopy in biological studies of cells,the technology is rarely used to monitor functional changes in live cells.By combining fast spinning disc-confocal structur...
关键词：super-resolution imaging ER Ca2+release MITOCHONDRIAL sequential response 
Identification of mecciRNAs and their roles in the mitochondrial entry of proteins被引量：19
《Science China(Life Sciences)》2020年第10期1429-1449,共21页Xu Liu Xiaolin Wang Jingxin Li Shanshan Hu Yuqi Deng Hao Yin Xichen Bao Qiangfeng Cliff Zhang Geng Wang Baolong Wang Qinghua Shi Ge Shan 
Supported by grants to G. S.: the National Key R&D Program of China (2019YFA0802600 and 2018YFC1004500);the National Natural Science Foundation of China (31725016, 31930019, and 91940303);and the Strategic Priority Research Program (Pilot Study) “Biological basis of aging and therapeutic strategies” of the Chinese Academy of Sciences (XDPB10).
Mammalian mitochondria have small genomes encoding very limited numbers of proteins.Over one thousand proteins and noncoding RNAs encoded by the nuclear genome must be imported from the cytosol into the mitochondria.H...
关键词：MITOCHONDRIA circRNA mecciRNA mitochondrial protein import 
Regulation of mitochondrial NAD pool via NAD^+ transporter 2 is essential for matrix NADH homeostasis and ROS production in Arabidopsis被引量：5
《Science China(Life Sciences)》2019年第8期991-1002,共12页Lilan Luo Yajun He Yannan Zhao Qian Xu Jian Wu Haiyan Ma Hongyan Guo Lin Bai Jianru Zuo Jian-Min Zhou Hong Yu Jiayang Li 
supported by the National Natural Science Foundation of China (31521001, 91854103, 31661143025)
Reactive oxygen species(ROS) play a crucial role in numerous biological processes in plants, including development, responses to environmental stimuli, and programmed cell death(PCD). Deficiency in MOSAIC DEATH 1(MOD1...
关键词：MITOCHONDRIAL NAD POOL NAD^+ TRANSPORTER 2 mtROS 
Metabolic characteristics and nutrient utilization in high-feedefficiency pigs selected using different feed conversion ratio models被引量：5
《Science China(Life Sciences)》2019年第7期959-970,共12页Beibei He Tiantian Li Wei Wang Hang Gao Yu Bai Shuai Zhang Jianjun Zang Defa Li Junjun Wang 
supported by the National Natural Science Foundation of China(31630074);the Beijing Municipal Natural Science Foundation(S170001);the National Key Research and Development Program of China(2016YFD0500506);the 111 Project(B16044);Jinxinnong Animal Science Developmental Foundation and Hunan Co-Innovation Center of Animal Production Safety
An understanding of the nutrient utilization characteristics of pigs with different feed efficiencies(FEs) will help us to develop new strategies to reduce the costs of pig production.In this study,we selected pigs wi...
关键词：feed efficiency CAECUM MICROBIOTA INTESTINAL functions MUSCLE growth and MITOCHONDRIAL activity growing PIGS 
Mitochondrial Ca^(2+) uptake in skeletal muscle health and disease被引量：4
《Science China(Life Sciences)》2016年第8期770-776,共7页Jingsong Zhou Kamal Dhakal Jianxun Yi 
supported by National Institute of Arthritis and Musculoskeletal and Skin Diseases(NIAMS)/National Institutes of Health(NIH)Grant(R01 AR057404)to Jingsong Zhou
Muscle uses Ca2＋ as a messenger to control contraction and relies on ATP to maintain the intracellular Ca2＋ homeostasis. Mi- tochondria are the major sub-cellular organelle of ATP production. With a negative inner m...
关键词：skeletal muscle MITOCHONDRIA CA2+ 
Life after the birth of the mitochondrial Na^+/Ca^(2+) exchanger,NCLX被引量：4
《Science China(Life Sciences)》2015年第1期59-65,共7页NITA Lulia I. HERSHFINKEL Michal SEKLER Israel 
Powered by the mitochondrial membrane potential,Ca2+ permeates the mitochondria via a Ca2+ channel termed Ca2+ uniporter and is pumped out by a Na+/Ca2+ exchanger,both of which are located on the inner mitochondrial m...
关键词：NCLX MCU mitochondrial Ca^2＋ signaling Na^＋/Ca^2＋ exchanger Na＋ signaling 
Mitochondrial genome sequences of Artemia tibetiana and Artemia urmiana:assessing molecular changes for high plateau adaptation被引量：5
《Science China(Life Sciences)》2013年第5期440-452,共13页ZHANG HangXiao LUO QiBin SUN Jing LIU Fei WU Gang YU Jun WANG WeiWei 
supported by the National Natural Science Foundation of China(30221004)to Wang WeiWei;the National Basic Research Program from Ministry of Science and Technology of China(2011CB944100)to Yu Jun
Brine shrimps,Artemia(Crustacea,Anostraca),inhabit hypersaline environments and have a broad geographical distribution from sea level to high plateaus.Artemia therefore possess significant genetic diversity,which give...
关键词：mitochondrial genome Artemia tibetiana sequence variation high plateau species 
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