2j8o: Difference between revisions
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==Overview== | ==Overview== | ||
Most of the structure of the giant muscle protein titin is formed by small, modular domains. Many of them are predicted to be arranged in repeats with, short linkers that may be key determinants of the peculiar elastic, properties of titin. Here, we present the molecular structure of a tandem, arrangement of two immunoglobulin-like domains, A168 and A169, located, within the A-band segment of titin. The two domains are connected by a 17, residue long beta-strand and form a common interface. Based on these data, we establish general principles to estimate the amount of conformational, flexibility of tandem domain motifs in titin. An unusual bulge within the, second domain, A169, is directly involved into binding to a sarcomeric, ligand, MURF-1, thus suggesting a dual role of this tandem for both the, mechanical properties of titin and for sarcomeric signaling. | Most of the structure of the giant muscle protein titin is formed by small, modular domains. Many of them are predicted to be arranged in repeats with, short linkers that may be key determinants of the peculiar elastic, properties of titin. Here, we present the molecular structure of a tandem, arrangement of two immunoglobulin-like domains, A168 and A169, located, within the A-band segment of titin. The two domains are connected by a 17, residue long beta-strand and form a common interface. Based on these data, we establish general principles to estimate the amount of conformational, flexibility of tandem domain motifs in titin. An unusual bulge within the, second domain, A169, is directly involved into binding to a sarcomeric, ligand, MURF-1, thus suggesting a dual role of this tandem for both the, mechanical properties of titin and for sarcomeric signaling. | ||
==Disease== | |||
Known diseases associated with this structure: Cardiomyopathy, dilated, 1G OMIM:[[http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=188840 188840]], Cardiomyopathy, familial hypertrophic OMIM:[[http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=188840 188840]], Muscular dystrophy, limb-girdle, type 2J OMIM:[[http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=188840 188840]], Myopathy, proximal, with early respiratory muscle involvement OMIM:[[http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=188840 188840]], Tibial muscular dystrophy, tardive OMIM:[[http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=188840 188840]] | |||
==About this Structure== | ==About this Structure== | ||
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[[Category: wd repeat]] | [[Category: wd repeat]] | ||
''Page seeded by [http://ispc.weizmann.ac.il/oca OCA ] on Mon Nov | ''Page seeded by [http://ispc.weizmann.ac.il/oca OCA ] on Mon Nov 12 22:53:00 2007'' | ||
