Research progress on the mitochondrial translocation and degradation

Abstract

Abstract: The mitochondrial proteins translocation and degradation coordinate to maintain the proteostasis network of mitochondria. Their dysfunction is one of the pathogenesis mechanims of metabolic diseases. The dysfunction of transportation and degradation prevents the nuclear-encoded mitochondrial protein from importing and cells from clearing abnormal proteins in time, which ultimately leads to mitochondrial damage. In the diabetic cardiomyocyte, decreased activity of electron transport chain (ETC), increased production of reactive oxygen species (ROS) and decreased energy productivity caused by disorders of mitochondrial transportation and degradation are main reasons for the development of diabetic cardiomyopathy (DCM).

Key words: mitochondrial translocation, mitochondrial degradation, diabetic cardiomyopathy

CLC Number:

R363

WANG Meng-yi, LI Qian-zhu, ZHANG Shi-wu, SHU Feng, ZHANG Wei-hua. Research progress on the mitochondrial translocation and degradation[J]. Basic & Clinical Medicine, 2021, 41(10): 1523-1528.

References

[1]Backes S, Hess S, Boos F, et al. Tom70 enhances mitochondrial preprotein import efficiency by binding to internal targeting sequences[J]. J Cell Biol, 2018, 217: 1369-1382.
[2]Araiso Y, Tsutsumi A, Qiu J, et al. Structure of the mitochondrial import gate reveals distinct preprotein paths[J]. Nature, 2019, 575: 395-401.
[3]Doan KN, Grevel A, Mårtensson CU, et al. The mitochondrial import complex MIM functions as main translo-case for α-helical outer membrane proteins[J]. Cell Rep, 2020, 31: 107567.doi:10.1016/j.cefrep.2020.107567.
[4]Caumont-Sarcos A, Moulin C, Poinot L, et al. Transmembrane coordination of preprotein recognition and motor coupling by the mitochondrial presequence receptor Tim50[J]. Cell Rep, 2020, 30: 3092-3104.
[5]Kumar A, Matta SK, D'Silva P. Role of conserved regions of Tim22 in the structural organization of the carrier translocase[J]. J Cell Sci, 2020.doi: 10.1242/jcs.244632.
[6]Kumar A, Matta SK, D'Silva P. Conserved regions of budding yeast Tim22 have a role in structural organization of the carrier translocase[J]. J Cell Sci, 2020, 133: jcs244632. doi: 10.1242/jcs.244632.
[7]Weinhäupl K, Lindau C, Hessel A, et al. Structural basis of membrane protein chaperoning through the mitochon-drial intermembrane space[J]. Cell, 2018, 175: 1365-1379.
[8]Sun Y, Teng Z, Sun X, et al. Exogenous H2S reduces the acetylation levels of mitochondrial respiratory enzymes via regulating the NAD+-SIRT3 pathway in cardiac tissues of db/db mice[J]. Am J Physiol Endocrinol Metab, 2019, 317: E284-E297.
[9]Rolland SG, Schneid S, Schwarz M, et al. Compromised mitochondrial protein import acts as a signal for UPRmt[J]. Cell Rep, 2019, 28: 1659-1669.
[10]Wrobel L, Topf U, Bragoszewski P, et al. Mistargeted mitochondrial proteins activate a proteostatic response in the cytosol[J]. Nature, 2015, 524: 485-488.
[11]Mårtensson CU, Priesnitz C, Song J, et al. Mitochondrial protein translocation-associated degradation[J]. Nature, 2019, 569: 679-683.
[12]Su T, Izawa T, Thoms M, et al. Structure and function of Vms1 and Arb1 in RQC and mitochondrial proteome homeostasis [J]. Nature, 2019, 570: 538-542.
[13]Matsumoto S, Nakatsukasa K, Kakuta C, et al. Msp1 clears mistargeted proteins by facilitating their transfer from mitochondria to the ER[J]. Mol Cell, 2019, 76: 191-205.
[14]Weidberg H, Amon A. mitoCPR-A surveillance pathway that protects mitochondria in response to protein import stress[J]. Science, 2018, 360. doi: 10.1126/science.aan4146.
[15]Pryde KR, Taanman JW, Schapira AH. A LON-ClpP proteolytic axis degrades complex I to extinguish ROS production in depolarized mitochondria[J]. Cell Rep, 2016, 17: 2522-2531.
[16]Bhaskaran S, Pharaoh G, Ranjit R, et al. Loss of mitochondrial protease ClpP protects mice from diet-induced obesity and insulin resistance[J]. EMBO Rep, 2018, 19: e45009. doi: 10.15252/embr.201745009.
[17]Yan C, Gong L, Chen L, et al. PHB2 (prohibitin 2) promotes PINK1-PRKN/Parkin-dependent mitophagy by the PARL-PGAM5-PINK1 axis[J]. Autophagy, 2020, 16: 419-434.
[18]Plun-Favreau H, Burchell VS, Holmström KM, et al. HtrA2 deficiency causes mitochondrial uncoupling through the F1F0-ATP synthase and consequent ATP depletion[J]. Cell Death Dis, 2012, 3: e335. doi: 10.1038/cddis.2012.77.
[19]Liu W, Duan X, Fang X, et al. Mitochondrial protein import regulates cytosolic protein homeostasis and neuronal integrity[J]. Autophagy, 2018, 14: 1293-1309.
[20]Wu H, Wang Y, Li W, et al. Deficiency of mitophagy receptor FUNDC1 impairs mitochondrial quality and aggravates dietary-induced obesity and metabolic syndrome[J]. Autophagy, 2019, 15: 1882-1898.
[21]尼加提·海热拉, 姚娟, 方舒, 等. 心肌钾离子通道KCNQ1基因rs760419多态性与新疆维吾尔族群体患心力衰竭的相关性[J]. 国际遗传学杂志, 2018,41: 86-92.
[22]刘英晓, 张莎莎, 毕丽妨. 线粒体型糖尿病合并MELAS综合征1例分析[J]. 国际遗传学杂志, 2019, 42: 437-439.
[23]Tong M, Saito T, Zhai P, et al. Mitophagy is essential for maintaining cardiac function during high fat diet-induced diabetic cardiomyopathy[J]. Circ Res, 2019, 124: 1360-1371.
[24]Kleinridders A, Lauritzen HP, Ussar S, et al. Leptin regulation of Hsp60 impacts hypothalamic insulin signaling[J]. J Clin Invest, 2013, 123: 4667-4680.
[25]Segref A, Kevei É, Pokrzywa W, et al. Pathogenesis of human mitochondrial diseases is modulated by reduced activity of the ubiquitin/proteasome system[J]. Cell Metab, 2014, 19: 642-652.