肌聯蛋白

肌聯蛋白
有效結構
PDB	直系同源檢索：PDBe, RCSB
PDB查詢代碼列表
	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
標識
代號	TTN; CMD1G; CMH9; CMPD4; EOMFC; HMERF; LGMD2J; MYLK5; TMD
擴展標識	遺傳學：188840 同源基因：130650 GeneCards: TTN Gene
EC編號	2.7.11.1
基因本體論描述
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;
細胞成分	· condensed nuclear chromosome; · extracellular region; · cytosol; · striated muscle thin filament; · Z disc; · M band; · I band;
生物過程	· 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表達模式
	更多表達數據
直系同源體
	閱; 論; 編;

肌聯蛋白（英語：titin），又稱肌巨蛋白，是人體中是由肌聯蛋白基因（TTN）編碼的蛋白質。^[1]^[2]肌聯蛋白是一個巨大的蛋白質，為肌肉收縮的彈性元件。它由244個結構域以及之間的肽序列連接組成。^[3]這些結構域在蛋白拉伸時去摺疊，而在張力去除後重新摺疊。^[4]

肌聯蛋白是已知最大的蛋白質^[5]，同時肌聯蛋白基因也擁有已知的單基因中最多的外顯子（363個）。^[6]

肌聯蛋白對橫紋肌的收縮很重要，它橫跨肌節從Z線到M線的區域，同粗肌絲的裝配和位置固定有關。在這個基因的突變能引起肌肉疾病，例如肢帶型肌營養不良。^[7]

肌聯蛋白有已知最長的IUPAC命名（如氧的IUPAC命名是oxygen）有189819個字母長。^[8]

另見

名稱獨特的化學物質列表

參考文獻

↑ 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. 請檢查|date=中的日期值 (幫助)
↑ 18萬個字母

[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. 請檢查|date=中的日期值 (幫助)

[8] 18萬個字母

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