肌联蛋白

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肌联蛋白
有效结构
PDB 直系同源检索:PDBe, RCSB
标识
代号 TTN; CMD1G; CMH9; CMPD4; EOMFC; HMERF; LGMD2J; MYLK5; TMD
扩展标识 遗传学188840 鼠基因98864 同源基因130650 GeneCards: TTN Gene
EC編號 2.7.11.1
RNA表达模式
PBB GE TTN 208195 at tn.png
更多表达数据
直系同源体
物种 人类 鼠类
Entrez 7273 22138
Ensembl ENSG00000155657 ENSMUSG00000051747
UniProt Q8WZ42 A2ASS6
mRNA序列 NM_001256850.1 NM_011652.3
蛋白序列 NP_001243779.1 NP_035782.3
基因位置 Chr 2:
179.39 – 179.7 Mb
Chr 2:
76.7 – 76.98 Mb
PubMed查询 [1] [2]

肌联蛋白英语titin)在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Z线M线的区域,同粗肌丝的装配和位置固定有关。

参考文献[编辑]

  1. ^ Entrez Gene: TTN titin. 
  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.May, 345 (6272): 273–6. doi:10.1038/345273a0. PMID 2129545. 
  3. ^ Labeit S, Kolmerer B. Titins: giant proteins in charge of muscle ultrastructure and elasticity. Science. 1995.October, 270 (5234): 293–6. doi:10.1126/science.270.5234.293. PMID 7569978. 
  4. ^ Minajeva A, Kulke M, Fernandez JM, Linke WA. Unfolding of titin domains explains the viscoelastic behavior of skeletal myofibrils. Biophys. J. 2001.March, 80 (3): 1442–51. doi:10.1016/S0006-3495(01)76116-4. PMC 1301335. PMID 11222304. 
  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.October, 100 (22): 12688–93. doi:10.1073/pnas.2133733100. PMC 240679. PMID 14563922. 
  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.November, 89 (11): 1065–72. doi:10.1161/hh2301.100981. PMID 11717165.