2j8h

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spinqualitylabelsall models 2j8h, resolution 1.99Å Show:Asymmetric Unit Biological Assembly Drag the structure with the mouse to rotate   Export Animated Image

STRUCTURE OF THE IMMUNOGLOBULIN TANDEM REPEAT A168-A169 OF TITIN

Contents 1 Overview 2 Disease 3 About this Structure 4 Reference

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.

Disease

Known diseases associated with this structure: Cardiomyopathy, dilated, 1G OMIM:[188840], Cardiomyopathy, familial hypertrophic OMIM:[188840], Muscular dystrophy, limb-girdle, type 2J OMIM:[188840], Myopathy, early-onset, with fatal cardiomyopathy OMIM:[188840], Myopathy, proximal, with early respiratory muscle involvement OMIM:[188840], Tibial muscular dystrophy, tardive OMIM:[188840]

About this Structure

2J8H is a Single protein structure of sequence from Homo sapiens with as ligand. Active as Non-specific serine/threonine protein kinase, with EC number 2.7.11.1 Known structural/functional Site: . Full crystallographic information is available from OCA.

Reference

Rigid conformation of an immunoglobulin domain tandem repeat in the A-band of the elastic muscle protein titin., Muller S, Lange S, Gautel M, Wilmanns M, J Mol Biol. 2007 Aug 10;371(2):469-80. Epub 2007 May 25. PMID:17574571

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