Titin (TTN) Human Recombinant Protein

Key component in the assembly and functioning of vertebrate striated muscles. By providing connections at the level of individual microfilaments, it contributes to the fine balance of forces between the two halves of the sarcomere. The size and extensibility of the cross-links are the main determinants of sarcomere extensibility properties of muscle. In non-muscle cells, ses to play a role in chromosome condensation and chromosome segregation during mitosis. Might link the lamina network to chromatin or nuclear actin, or both during interphase.

Titins, giant proteins in charge of muscle ultrastructure and elasticity.Labeit S., Kolmerer B.Science 270:293-296(1995) Series of exon-skipping events in the elastic spring region of titin as the structural basis for myofibrillar elastic diversity.Freiburg A., Trombitas K., Hell W., Cazorla O., Fougerousse F., Centner T., Kolmerer B., Witt C., Beckmann J.S., Gregorio C.C., Granzier H., Labeit S.Circ. Res. 86:1114-1121(2000) The complete gene sequence of titin, expression of an unusual ~700 kDa titin isoform and its interaction with obscurin identify a novel Z-line to I-band linking system.Bang M.-L., Centner T., Fornoff F., Geach A.J., Gotthardt M., McNabb M., Witt C.C., Labeit D., Gregorio C.C., Granzier H., Labeit S.Circ. Res. 89:1065-1072(2001) Generation and annotation of the DNA sequences of human chromosomes 2 and 4.Hillier L.W., Graves T.A., Fulton R.S., Fulton L.A., Pepin K.H., Minx P., Wagner-McPherson C., Layman D., Wylie K., Sekhon M., Becker M.C., Fewell G.A., Delehaunty K.D., Miner T.L., Nash W.E., Kremitzki C., Oddy L., Du H., Sun H., Bradshaw-Cordum H., Ali J., Carter J., Cordes M., Harris A., Isak A., van Brunt A., Nguyen C., Du F., Courtney L., Kalicki J., Ozersky P., Abbott S., Armstrong J., Belter E.A., Caruso L., Cedroni M., Cotton M., Davidson T., Desai A., Elliott G., Erb T., Fronick C., Gaige T., Haakenson W., Haglund K., Holmes A., Harkins R., Kim K., Kruchowski S.S., Strong C.M., Grewal N., Goyea E., Hou S., Levy A., Martinka S., Mead K., McLellan M.D., Meyer R., Randall-Maher J., Tomlinson C., Dauphin-Kohlberg S., Kozlowicz-Reilly A., Shah N., Swearengen-Shahid S., Snider J., Strong J.T., Thompson J., Yoakum M., Leonard S., Pearman C., Trani L., Radionenko M., Waligorski J.E., Wang C., Rock S.M., Tin-Wollam A.-M., Maupin R., Latreille P., Wendl M.C., Yang S.-P., Pohl C., Wallis J.W., Spieth J., Bieri T.A., Berkowicz N., Nelson J.O., Osborne J., Ding L., Meyer R., Sabo A., Shotland Y., Sinha P., Wohldmann P.E., Cook L.L., Hickenbotham M.T., Eldred J., Williams D., Jones T.A., She X., Ciccarelli F.D., Izaurralde E., Taylor J., Schmutz J., Myers R.M., Cox D.R., Huang X., McPherson J.D., Mardis E.R., Clifton S.W., Warren W.C., Chinwalla A.T., Eddy S.R., Marra M.A., Ovcharenko I., Furey T.S., Miller W., Eichler E.E., Bork P., Suyama M., Torrents D., Waterston R.H., Wilson R.K.Nature 434:724-731(2005) The central Z-disk region of titin is assembled from a novel repeat in variable copy numbers.Gautel M., Goulding D., Bullard B., Weber K., Furst D.O.J. Cell Sci. 109:2747-2754(1996) Dissecting titin into its structural motifs identification of an alpha helix near the N-terminus.Musco G., Tziatzos C., Schuck P., Pastore A.Biochemistry 34:553-561(1995) Familial dilated cardiomyopathy locus maps to chromosome 2q31.Siu B.L., Niimura H., Osborne J.A., Fatkin D., MacRae C., Solomon S., Benson D.W., Seidman J.G., Seidman C.E.Circulation 99:1022-1026(1999) Species variations in cDNA sequence and exon splicing patterns in the extensible I-band region of cardiac titin relation to passive tension.Greaser M.L., Berri M., Warren C.M., Mozdziak P.E.J. Muscle Res. Cell Motil. 23:473-482(2002) Lubec G., Chen W.-Q., Sun Y. Titin antibodies in myasthenia gravis identification of a major immunogenic region of titin.Gautel M., Lakey A., Barlow D.P., Holmes Z., Scales S., Leonard K., Labeit S., Mygland A., Gilhus N.E., Aarli J.A.Neurology 43:1581-1585(1993) Towards a molecular understanding of titin.Labeit S., Gautel M., Lakey A., Trinick J.EMBO J. 11:1711-1716(1992) Phosphorylation of KSF motifs in the C-terminal region of titin in differentiating myoblasts.Gautel M., Leonard K., Labeit S.EMBO J. 12:3827-3834(1993) The full-ORF clone resource of the German cDNA consortium.Bechtel S., Rosenfelder H., Duda A., Schmidt C.P., Ernst U., Wellenreuther R., Mehrle A., Schuster C., Bahr A., Bloecker H., Heubner D., Hoerlein A., Michel G., Wedler H., Koehrer K., Ottenwaelder B., Poustka A., Wiemann S., Schupp I.BMC Genomics 8:399-399(2007) Two immunoglobulin-like domains of the Z-disc portion of titin interact in a conformation-dependent way with telethonin.Mues A., van der Ven P.F.M., Young P., Furst D.O., Gautel M.FEBS Lett. 428:111-114(1998) Interaction of nebulin SH3 domain with titin PEVK and myopalladin implications for the signaling and assembly role of titin and nebulin.Ma K., Wang K.FEBS Lett. 532:273-278(2002) Subcellular targeting of metabolic enzymes to titin in heart muscle may be mediated by DRAL/FHL-2.Lange S., Auerbach D., McLoughlin P., Perriard E., Schafer B.W., Perriard J.-C., Ehler E.J. Cell Sci. 115:4925-4936(2002) The hydrophilic domain of small ankyrin-1 interacts with the two N-terminal immunoglobulin domains of titin.Kontrogianni-Konstantopoulos A., Bloch R.J.J. Biol. Chem. 278:3985-3991(2003) The muscle ankyrin repeat proteins CARP, ankrd2/Arpp and DARP as a family of titin filament-based stress response molecules.Miller M.K., Bang M.-L., Witt C.C., Labeit D., Trombitas C., Watanabe K., Granzier H., McElhinny A.S., Gregorio C.C., Labeit S.J. Mol. Biol. 333:951-964(2003) Association of the chaperone alphaB-crystallin with titin in heart muscle.Bullard B., Ferguson C., Minajeva A., Leake M.C., Gautel M., Labeit D., Ding L., Labeit S., Horwitz J., Leonard K.R., Linke W.A.J. Biol. Chem. 279:7917-7924(2004) MURF-1 and MURF-2 target a specific subset of myofibrillar proteins redundantly towards understanding MURF-dependent muscle ubiquitination.Witt S.H., Granzier H., Witt C.C., Labeit S.J. Mol. Biol. 350:713-722(2005) Nuclear titin interacts with A- and B-type lamins in vitro and in vivo.Zastrow M.S., Flaherty D.B., Benian G.M., Wilson K.L.J. Cell Sci. 119:239-249(2006) Mechanical stress-strain sensors embedded in cardiac cytoskeleton Z disk, titin, and associated structures.Hoshijima M.Am. J. Physiol. 290:H1313-H1325(2006) Interactions with M-band titin and calpain 3 link myospryn (CMYA5) to tibial and limb-girdle muscular dystrophies.Sarparanta J., Blandin G., Charton K., Vihola A., Marchand S., Milic A., Hackman P., Ehler E., Richard I., Udd B.J. Biol. Chem. 285:30304-30315(2010) Tertiary structure of an immunoglobulin-like domain from the giant muscle protein titin a new member of the I set.Pfuhl M., Pastore A.Structure 3:391-401(1995) Structural basis for activation of the titin kinase domain during myofibrillogenesis.Mayans O., van der Ven P.F.M., Wilm M., Mues A., Young P., Furst D.O., Wilmanns M., Gautel M.Nature 395:863-869(1998) The three-dimensional structure of a type I module from titin a prototype of intracellular fibronectin type III domains.Goll C.M., Pastore A., Nilges M.Structure 6:1291-1302(1998) Structural evidence for a possible role of reversible disulphide bridge formation in the elasticity of the muscle protein titin.Mayans O., Wuerges J., Canela S., Gautel M., Wilmanns M.Structure 9:331-340(2001) Palindromic assembly of the giant muscle protein titin in the sarcomeric Z-disk.Zou P., Pinotsis N., Lange S., Song Y.-H., Popov A., Mavridis I., Mayans O.M., Gautel M., Wilmanns M.Nature 439:229-233(2006) Structural analysis of the titin gene in hypertrophic cardiomyopathy identification of a novel disease gene.Satoh M., Takahashi M., Sakamoto T., Hiroe M., Marumo F., Kimura A.Biochem. Biophys. Res. Commun. 262:411-417(1999) Tibial muscular dystrophy is a titinopathy caused by mutations in TTN, the gene encoding the giant skeletal-muscle protein titin.Hackman P., Vihola A., Haravuori H., Marchand S., Sarparanta J., De Seze J., Labeit S., Witt C., Peltonen L., Richard I., Udd B.Am. J. Hum. Genet. 71:492-500(2002) Titin mutations as the molecular basis for dilated cardiomyopathy.Itoh-Satoh M., Hayashi T., Nishi H., Koga Y., Arimura T., Koyanagi T., Takahashi M., Hohda S., Ueda K., Nouchi T., Hiroe M., Marumo F., Imaizumi T., Yasunami M., Kimura A.Biochem. Biophys. Res. Commun. 291:385-393(2002) Mutations of TTN, encoding the giant muscle filament titin, cause familial dilated cardiomyopathy.Gerull B., Gramlich M., Atherton J., McNabb M., Trombitas K., Sasse-Klaassen S., Seidman J.G., Seidman C., Granzier H., Labeit S., Frenneaux M., Thierfelder L.Nat. Genet. 30:201-204(2002) Tibial muscular dystrophy in a Belgian family.Van den Bergh P.Y.K., Bouquiaux O., Verellen C., Marchand S., Richard I., Hackman P., Udd B.Ann. Neurol. 54:248-251(2003) Functional analysis of titin/connectin N2-B mutations found in cardiomyopathy.Matsumoto Y., Hayashi T., Inagaki N., Takahashi M., Hiroi S., Nakamura T., Arimura T., Nakamura K., Ashizawa N., Yasunami M., Ohe T., Yano K., Kimura A.J. Muscle Res. Cell Motil. 26:367-374(2005) The kinase domain of titin controls muscle gene expression and protein turnover.Lange S., Xiang F., Yakovenko A., Vihola A., Hackman P., Rostkova E., Kristensen J., Brandmeier B., Franzen G., Hedberg B., Gunnarsson L.G., Hughes S.M., Marchand S., Sejersen T., Richard I., Edstroem L., Ehler E., Udd B., Gautel M.Science 308:1599-1603(2005) C-terminal titin deletions cause a novel early-onset myopathy with fatal cardiomyopathy.Carmignac V., Salih M.A.M., Quijano-Roy S., Marchand S., Al Rayess M.M., Mukhtar M.M., Urtizberea J.A., Labeit S., Guicheney P., Leturcq F., Gautel M., Fardeau M., Campbell K.P., Richard I., Estournet B., Ferreiro A.Ann. Neurol. 61:340-351(2007) Patterns of somatic mutation in human cancer genomes.Greenman C., Stephens P., Smith R., Dalgliesh G.L., Hunter C., Bignell G., Davies H., Teague J., Butler A., Stevens C., Edkins S., O'Meara S., Vastrik I., Schmidt E.E., Avis T., Barthorpe S., Bhamra G., Buck G., Choudhury B., Clements J., Cole J., Dicks E., Forbes S., Gray K., Halliday K., Harrison R., Hills K., Hinton J., Jenkinson A., Jones D., Menzies A., Mironenko T., Perry J., Raine K., Richardson D., Shepherd R., Small A., Tofts C., Varian J., Webb T., West S., Widaa S., Yates A., Cahill D.P., Louis D.N., Goldstraw P., Nicholson A.G., Brasseur F., Looijenga L., Weber B.L., Chiew Y.-E., DeFazio A., Greaves M.F., Green A.R., Campbell P., Birney E., Easton D.F., Chenevix-Trench G., Tan M.-H., Khoo S.K., Teh B.T., Yuen S.T., Leung S.Y., Wooster R., Futreal P.A., Stratton M.R.Nature 446:153-158(2007) Diagnostic exome sequencing in persons with severe intellectual disability.de Ligt J., Willemsen M.H., van Bon B.W., Kleefstra T., Yntema H.G., Kroes T., Vulto-van Silfhout A.T., Koolen D.A., de Vries P., Gilissen C., del Rosario M., Hoischen A., Scheffer H., de Vries B.B., Brunner H.G., Veltman J.A., Vissers L.E.N. Engl. J. Med. 367:1921-1929(2012) +Additional computationally mapped references.<p>Provides general information on the entry.

Small volumes of TTN recombinant protein vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

MBS1265279 is a recombinant protein targeting TTN. Also known as Recombinant Human Titin. Host: E Coli or Yeast or Baculovirus or Mammalian Cell.