肌联蛋白

肌联蛋白
有效结构
PDB	直系同源检索：PDBe, RCSB
PDB查询代码列表
	1BPV, 1G1C, 1NCT, 1NCU, 1TIT, 1TIU, 1TKI, 1TNM, 1TNN, 1WAA, 1YA5, 2A38, 2BK8, 2F8V, 2ILL, 2J8H, 2J8O, 2NZI, 2RQ8, 2WP3, 2WWK, 2WWM, 2Y9R, 3KNB, 3LCY, 3LPW, 3PUC, 3Q5O, 3QP3
标识
代号	TTN; CMD1G; CMH9; CMPD4; EOMFC; HMERF; LGMD2J; MYLK5; TMD
扩展标识	遗传学：188840 同源基因：130650 GeneCards: TTN Gene
EC编号	2.7.11.1
基因本体论描述
分子功能	· protease binding; · nucleic acid binding; · protein serine/threonine kinase activity; · protein tyrosine kinase activity; · calcium ion binding; · protein binding; · calmodulin binding; · ATP binding; · structural constituent of muscle; · enzyme binding; · telethonin binding; · identical protein binding; · actinin binding; · protein self-association; · actin filament binding; · muscle alpha-actinin binding;
细胞成分	· condensed nuclear chromosome; · extracellular region; · cytosol; · striated muscle thin filament; · Z disc; · M band; · I band;
生物过程	· platelet degranulation; · muscle contraction; · striated muscle contraction; · mitotic chromosome condensation; · blood coagulation; · muscle filament sliding; · platelet activation; · skeletal muscle thin filament assembly; · skeletal muscle myosin thick filament assembly; · sarcomere organization; · regulation of protein kinase activity; · cardiac muscle fiber development; · sarcomerogenesis; · regulation of catalytic activity; · response to calcium ion; · cardiac myofibril assembly; · cardiac muscle tissue morphogenesis;
	Sources: Amigo / QuickGO
RNA表达模式
	更多表达数据
直系同源体
	查; 论; 编;

肌联蛋白（英语：titin），又称肌巨蛋白，是人体中是由肌联蛋白基因（TTN）编码的蛋白质。^[1]^[2]肌联蛋白是一个巨大的蛋白质，为肌肉收缩的弹性元件。它由244个结构域以及之间的肽序列连接组成。^[3]这些结构域在蛋白拉伸时去折叠，而在张力去除后重新折叠。^[4]

肌联蛋白是已知最大的蛋白质^[5]，同时肌联蛋白基因也拥有已知的单基因中最多的外显子（363个）。^[6]

肌联蛋白对横纹肌的收缩很重要，它横跨肌节从Z线到M线的区域，同粗肌丝的装配和位置固定有关。在这个基因的突变能引起肌肉疾病，例如肢带型肌营养不良。^[7]

肌联蛋白有已知最长的IUPAC命名（如氧的IUPAC命名是oxygen）有189819个字母长。^[8]

另见

名称独特的化学物质列表

参考文献

↑ Entrez Gene: TTN titin.
↑ Labeit S, Barlow DP, Gautel M, Gibson T, Holt J, Hsieh CL, Francke U, Leonard K, Wardale J, Whiting A. A regular pattern of two types of 100-residue motif in the sequence of titin. Nature. 1990-05, 345 (6272): 273–6. PMID 2129545. doi:10.1038/345273a0.
↑ Labeit S, Kolmerer B. Titins: giant proteins in charge of muscle ultrastructure and elasticity. Science. 1995-10, 270 (5234): 293–6. PMID 7569978. doi:10.1126/science.270.5234.293.
↑ Minajeva A, Kulke M, Fernandez JM, Linke WA. Unfolding of titin domains explains the viscoelastic behavior of skeletal myofibrils. Biophys. J. 2001-03, 80 (3): 1442–51. PMC 1301335 . PMID 11222304. doi:10.1016/S0006-3495(01)76116-4.
↑ Opitz CA, Kulke M, Leake MC, Neagoe C, Hinssen H, Hajjar RJ, Linke WA. Damped elastic recoil of the titin spring in myofibrils of human myocardium. Proc. Natl. Acad. Sci. U.S.A. 2003-10, 100 (22): 12688–93. PMC 240679 . PMID 14563922. doi:10.1073/pnas.2133733100.
↑ Bang ML, Centner T, Fornoff F, Geach AJ, Gotthardt M, McNabb M, Witt CC, Labeit D, Gregorio CC, Granzier H, Labeit S. The complete gene sequence of titin, expression of an unusual approximately 700-kDa titin isoform, and its interaction with obscurin identify a novel Z-line to I-band linking system. Circ. Res. 2001-11, 89 (11): 1065–72. PMID 11717165. doi:10.1161/hh2301.100981.
↑ Finsterer J. Klinik und Genetik der Gliedergürteldystrophien. Nervenarzt. December, 75 (12): 1153–66. doi:10.1007/s00115-004-1769-5. 请检查|date=中的日期值 (帮助)
↑ 18万个字母

[Entrez_7273-1] Entrez Gene: TTN titin.

[pmid2129545-2] Labeit S, Barlow DP, Gautel M, Gibson T, Holt J, Hsieh CL, Francke U, Leonard K, Wardale J, Whiting A. A regular pattern of two types of 100-residue motif in the sequence of titin. Nature. 1990-05, 345 (6272): 273–6. PMID 2129545. doi:10.1038/345273a0.

[pmid7569978-3] Labeit S, Kolmerer B. Titins: giant proteins in charge of muscle ultrastructure and elasticity. Science. 1995-10, 270 (5234): 293–6. PMID 7569978. doi:10.1126/science.270.5234.293.

[pmid11222304-4] Minajeva A, Kulke M, Fernandez JM, Linke WA. Unfolding of titin domains explains the viscoelastic behavior of skeletal myofibrils. Biophys. J. 2001-03, 80 (3): 1442–51. PMC 1301335 . PMID 11222304. doi:10.1016/S0006-3495(01)76116-4.

[pmid14563922-5] Opitz CA, Kulke M, Leake MC, Neagoe C, Hinssen H, Hajjar RJ, Linke WA. Damped elastic recoil of the titin spring in myofibrils of human myocardium. Proc. Natl. Acad. Sci. U.S.A. 2003-10, 100 (22): 12688–93. PMC 240679 . PMID 14563922. doi:10.1073/pnas.2133733100.

[pmid11717165-6] Bang ML, Centner T, Fornoff F, Geach AJ, Gotthardt M, McNabb M, Witt CC, Labeit D, Gregorio CC, Granzier H, Labeit S. The complete gene sequence of titin, expression of an unusual approximately 700-kDa titin isoform, and its interaction with obscurin identify a novel Z-line to I-band linking system. Circ. Res. 2001-11, 89 (11): 1065–72. PMID 11717165. doi:10.1161/hh2301.100981.

[Gliedergürteldystrophie-7] Finsterer J. Klinik und Genetik der Gliedergürteldystrophien. Nervenarzt. December, 75 (12): 1153–66. doi:10.1007/s00115-004-1769-5. 请检查|date=中的日期值 (帮助)

[8] 18万个字母

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